bluuebunny/crossref_metadata_2025 huggingface dataset:
Isomerism of the left atrial appendages associated with bronchoatrial discordance and unusual systemic venous drainage
Authors: Jewel Francis M. , Joffe Hyam S. , Wilde Peter
Published: August 2008

A complex case of congenital heart disease is presented which features isomerism of the left atrial appendages combined with bronchoatrial discordance and an anteriorly placed non-interrupted inferior caval vein draining to the left-sided atrium. This combination of anomalies has, as far as we are aware, not been described before. It emphasizes the importance of direct confirmation of atrial arrangement by atrial angiography or transesophageal echocardiography.
Neutrophil adhesion and the inflammatory response induced by cardiopulmonary bypass
Authors: Finn Adam , Dreyer William J.
Published: August 2008

The activation of inflammatory systems as a consequence of cardiopulmonary bypass represents one possible means by which inj ury to tissues and associated dysfunction of organs may occur in the postoperative cardiac patient, both pediatric and adult. Kirklin and colleagues demonstrated in the early 1980's that complement activation occurs as a consequence of blood exposure to the extracorporeal circuit. Subsequent studies demonstrated this to be true in systems using both bubble and membrane oxygenators. The anaphylatoxin C5a and its metabolite C5a des arg are produced during activation of the complement cascade. They are potent stimulants which may induce activation of neutrophils. This activation may be manifested as changes in shape, decreased cellular deformability, and changes in adhesive and secretory function. Activation of neutrophils by these fragments of complement could contribute to neutrophilmediated damage to tissues by promoting retention of neutrophils in vascular beds and secretion of cytotoxic substances. Recent studies demonstrating the release of neutrophil granular enzymes into the plasma following cardiopulmonary bypass are evidence that activation of neutrophils is taking place in this setting.
Fetal responses to extracorporeal circulatory support
Authors: Hanley Frank L.
Published: August 2008

The current practice of cardiac surgery would be impossible without extracorporeal circulatory support. Although significant morbidity is still associated with these techniques of support, they are “tolerated” because the flexibility and practical advantages which they provide in clinical practice far outweigh the associated morbidity.
Echocardiography of the prominent Eustachian valve—associations and complications with cyanosis and right heart obstruction
Authors: Brook Michael M. , Silverman Norman H.
Published: August 2008

We reviewed the echocardiographic features of 16 children with prominent Eustachian valves, six of whom had associated pathology of the right heart, while eight had other anomalies, primarily atrial and ventricular septal defects. The hearts of two patients were structurally normal. Prolapse of the membrane through the orifice of the tricuspid valve or through the atrial septum was present in seven patients, two of whom were symptomatic. One presented with severe cyanosis which resolved after surgical removal on the basis of the echocardiographic findings. The other presented with atrial arrhythmias which did not improve with removal. The size or behavior of the membrane, including prolapse, did not correlate with either age or presence of pathology of the right heart. The echocardiographic appearance of prominent Eustachian valves is variable. Rarely, when prominent, the valves can cause symptoms associated with obstruction to the right heart, particularly cyanosis or an arrhythmia.
Smith-Magenis syndrome (interstitial deletion of chromosome 17p) and congenital heart disease
Authors: Abuelo Dianne N. , Corwin Robert D.
Published: August 2008

A syndrome with characteristic facial and skeletal features resulting from a specific deletion of material on the short arm of chromosome 17 was first described by Smith et al in 1982.1 The common findings are brachycephaly, hypoplasia of the middle face, and short broad hands associated with mental retardation. More than 50 cases have been reported, and about one third have congenital heart disease. We report here a patient with atrial and ventricular septal defects together with pulmonary valvar stenosis.
The role of balloon dilation of native aortic coarctation in neonates, infants and children
Authors: Galal Omar , Halees Zohair Al , Qureshi Shakeel , Gometza Begonia , Fawzy Mohammed Eid , Fadley Fadel Al , Dunn Bruce , Duran Carlos
Published: August 2008

In 38 children with aortic coarctation between the age of three weeks and 16 years, 44 balloon dilation procedures were attempted. At initial catheterization (n=38), the mean gradient dropped from 49.6±21.2 mm Hg prior to dilation, to 15.3±15.8 mm Hg after the procedure (p>0.05). Twenty-six patients were recatheterized 3–24 months after the initial procedure. The mean gradient remained low at 12.7±15.2 mm Hg (range 0–64). Six of the 26 (23%) patients required a second dilation because of significant recoarctation. This proved successful in four, but in two patients, the second dilation failed, and they were referred for elective surgery. Thus, good overall results were obtained in 24 of the 26 (92.3%) patients undergoing reinvestigation following balloon dilation. Complications included requirements for blood transfusion in 9/44 (20%), chest pain during balloon inflation in 3/44 (6.8%), rupture of the balloon in 3/44 (6.8%), and femoral arterial thrombectomy in 3/44 (6.8%). At a mean clinical follow-up of 21 months, 21% of the patients had a weaker femoral pulse on the side used for balloon dilation, while 21% had mild hypertension measured in the arms despite the absence of a significant gradient. One patient (2.6%) developed an aortic aneurysm as a complication of the procedure. We conclude that balloon dilation is a safe and effective procedure in the majority of the patients with native aortic coarctation.
Profile of aortopulmonary window in India—a study of twenty cases
Authors: Pinto Robin J. , Bhagwat Ajit R. , Loya Yunus S. , Sharma Satyavan
Published: August 2008

Twenty cases of aortopulmonary window are reported with special reference to some unusual modes of presentation and hemodynamics. Patients over 15 years of age accounted for 35% of the group. This delayed presentation may relate to referral patterns and socioeconomic conditions prevalent in India. Curiously, this elderly subset had two modes of presentation—either as large left-to-right shunts with low pulmonary vascular resistance (71.4%), or as Eisenmenger's syndrome (28.6%). The reasons for the low pulmonary vascular resistance in the majority of these older patients remain unclear. The echocardiographic features of these defects can be missed in presence of significant associated cardiac lesions. A careful search should, therefore, be made for an additional aortopulmonary window in all cases with evidence of intercirculatory shunting.
Left isomerism with absence of the spleen and obstructed pulmonary venous drainage—an uncommon association
Authors: Zimarino Marco , Gerboni Salvatore , Picchio Fernando M.
Published: August 2008

Cardiac and extracardiac anomalies tend to follow characteristic patterns when there is an isomeric arrangement of the heart and lungs. We present a case diagnosed correctly during prenatal life as having isomerism of the left atrial appendages in which some of the associated features were most unexpected, namely absence of the spleen and obstructed totally anomalous pulmonary venous connection. This association proved misleading for us in the management of the patient. We found it difficult to believe our findings in the light of our past knowledge, and this resulted in a delay in the referral for surgery. The final outcome was unfortunately fatal. In the management of isomerism of any type, therefore, a wide spectrum of combinations and “crossover” of cardiac anomalies must be anticipated, emphasizing the need to assess individually the arrangement of the different organs of the body.
An unusual cause of cyanosis after the modified Fontan procedure—closure of venous communications between the coronary sinus and left atrium by transcatheter techniques
Authors: Hayes Alison M. , Burrows Patricia E. , Benson Lee N.
Published: August 2008

Communication of the coronary sinus with the left atrium is an unusual anomaly. Two cases of tricuspid atresia are described where systemic desaturation developed following a modified Fontan procedure. In both, investigation demonstrated a communication between the coronary sinus and the left atrium. Transcatheter closure of these communications was achieved with a ductal device in one case and coil embolization in the other. Following occlusion and elimination of the right-to-left shunt, both patients became fully saturated in room air.
An unusual presentation of isolation of the right subclavian artery
Authors: Smith Julian A. , Hirschklau Michael J. , Reitz Bruce A.
Published: August 2008

Isolation of the right subclavian artery from the aortic arch is a rare malformation. When isolated, the artery originates from the right pulmonary artery via a right-sided arterial duct, which may be patent or closed at the time of presentation. Intracardiac or other anomalies of the great vessels are commonly associated with this lesion. We present a case of isolation of the right subclavian artery which was initially diagnosed as persistent patency of a left-sided arterial duct. Surgical correction was eventually achieved by disconnecting the right subclavian artery from the right pulmonary artery and translocating it to the ascending aorta.
The size of the coronary arteries in children with complete transposition before and after the arterial switch operation
Authors: Yatsunami Koichi , Nakazawa Makoto , Seguchi Masashi , Momma Kazuo , Imai Yasuharu
Published: August 2008

The size of the coronary arteries parallels the ventricular mass, thus it may be abnormal in complete transposition beyond infancy and could be influenced by the arterial switch operation. To investigate this possibility, we measured the diameters of the right, left main trunk, anterior descending, and circumflex coronary arteries before and three to seven years (mean 4.8) after the arterial switch operation in 17 patients with a “normal” distribution of the coronary arteries (so-called Shaher type 1). The values were compared with 18 controls who had Kawasaki disease with no apparent coronary arterial disease. The right, left anterior descending, and circumflex arteries were smaller than control values before the operation. The post-/preoperative ratios of the diameter were 1.16±0.11 for the right coronary artery, 1.18±0.16 for the left main trunk, 1.20±0.18 for the left anterior descending artery, and 1.22±0.26 for the circumflex artery. There were no significant differences among these values. After surgery, the right coronary artery was larger, but the left coronary arteries were smaller in the patients than in the controls: 2.5±0.3 vs 2.0±0.2 mm for the right coronary artery; 2.4±0.3 vs 2.7±0.1 mm for the left main trunk; 1.9±0.2 vs 2.4±0.2 mm for the left anterior descending artery; 1.6±0.4 vs 2.2±0.5 mm for the circumflex artery, respectively. The posterior descending coronary artery originated from the right coronary artery in all patients. The total cross-sectional area of the right coronary, left anterior descending, and circumflex arteries was 9.7±2.4 mm2in the patients, and 11.8±2.9 mm2in the controls (p>O. 1), suggesting that the increased size of the right coronary artery compensates for the small left coronary arteries. We conclude that the arterial system in complete transposition, with a large right coronary artery and small left coronary system, remains smaller than normal even at midterm follow-up after anatomic repair despite normalization of left ventricular volume and muscle mass.
Application of ice water to the face during controlled respiration—a measure of basal vagal tone
Authors: Yum Myung Kul , Kim Seung Hwan , Jung Yeoung Geun
Published: August 2008

We examined the effectiveness of the application of ice water to the face during metronome-controlled respiration (15 breaths per minute) for vagalstimulation. We also examined the importance of basal vagal tone and sympathovagal interaction in determining the individual response to the stimulation. Fifty-three boys, aged 12 and 13, were included in this study. Vagal tone and sympathovagal interaction were assessed by power spectral analysis of the variability of the RR interval (heart rate). Basal heart rate, high frequency power, and low-to-high frequency power ratios were 81 ± 13(58–110 beats/min), 791 ± 1061(56–4161 m.sec2) and 1.08±1.22 (0.04–4.85) during controlled respiration. After application of ice water, 23 children developed frequent nodal escape beats due to severe sinus bradycardia. Minimum heart rate, high frequency power, and low to high power ratios changed to 42± 12 (19–72 beats/min), 1890±1882 (211–7258 m.sec2) and 0.64±0.43 (0.12–1.46). The increased ratio of high frequency power, maximum decrement in heart rate, and its percent after stimulation were 5.44±5.62 (0.63–24.26), 39±14 (10–81 beats/mm) and 47±15 (16–81%), respectively. The increased ratio of high frequency power was correlated with basal logarithmic high frequency power (r=−0.60, p=O.0004). Maximum heart rate decrement was correlated with basal logarithmic high frequency power (r=−0.60, p=O.OO18) and low-to-high frequency power ratio (r=0.27, p=O.O4). We conclude that application of ice water to the faceduring controlled respiration induces powerful vagal stimulation and bradycardia similar to, or even greater than, facial immersion in cold water. There is wide variability of individual response which can be explained by the magnitude of absolute basal vagal tone.
Experimental studies on the implantation of intravascular stents in puppies—potential use for aortic coarctation and pulmonary arterial stenosis
Authors: Ino Toshihiro , Shimazaki Shinjiro , Yabuta Keijiro , Okada Ryozo
Published: August 2008

We have investigated angiographically and histologically the vascular response to implantation of intravascular stents in normal puppies. Eight balloon-expandable stents were placed in six mongrel puppies (weight 6.4–10.5 kg) under anesthesia. Four stents were successfully placed in the pulmonary arteries and three in the aorta. In one case, the strut of the stent was hooked by a leaflet of the tricuspid valve at the time of placement and eventually had to be implanted in the inferior caval vein. Two puppies died, one from bleeding from the femoral artery and the other from thrombotic obstruction at the superior mesenteric artery, both after successful implantation. The former was due to repeat arterial puncture and the latter wasdue to failure to use aspirin. Angiographic studies showed satisfactory opacities of the branch arteries in all but one puppy. The diameter of the arteries prior to implantation was 7.5±1.5 mm. The diameters at the middle of the stents immediately after implantation and during the period of follow-up were 6.9±1.7mm and 6.9±2.1 mm, respectively. Histologic studies revealed that neogenic endothelial layers of 30 to 40 pm in thickness had formed over the struts of the stent without formation of thrombusthree weeks after implantation. Scanning electron microscopy revealed that the endothelial cells of the neoendothelial layer had almost identical characteristics to those of normal arteries. These results indicate that clinical application of this stent is feasible but that use of anticoagulants is mandatory during and after implantation in the treatment of selected patients with aortic coarctation or pulmonary arterial stenosis. Relative progression of stenosis may occur, however, and the inability for re-expansion is a major disadvantage of this stent.
Enlargement of a classical Blalock-Taussig shunt by implantation of a stent after failed balloon angioplasty
Authors: McLeod Karen , Blackburn Michael E. , Gibbs John L.
Published: August 2008

The period of adequate palliation from a Blalock-Taussig shunt may be limited by the development of stenosis of the shunt. Balloon dilatation oftenis unsuccessful due to recoil of the vessel. We report the case of a 22-year-old man with a stenosed Blalock-Taussig shunt who was successfully treated by the implantation of a balloon expandable stent.
Normal values for cardiac and great arterial dimensions in premature infants by cross-sectional echocardiography
Authors: Imai Toshiro , Satomi Gengi , Yasukochi Satoshi , Harada Yorikazu , Takeuchi Takamasa , Gotoh Hirohisa , Sakamoto Takahiko
Published: August 2008

The number of neonates who undergo intracardiac repair for congenital heart disease has increased in recent years. In considering appropriate surgical options in such circumstances, normal values for various cardiovascular dimensions are needed. We measured 19 parameters for the heart and great arteries (four valves, pulmonary tree, aortic arch, ascending and descending aorta, ventricular septum, left ventricular internal and posterior wall dimensions) using cross-sectional echocardiography in 55 premature and full-term infants (ranging in gestational age from 23 to 41 weeks, in body weight from 543 to 3966 g). The increase in each dimension correlated closely with body weight (r=0.62 to 0.87) and was found to follow a linear regression. The diameter of the isthmus was found to be smaller, and the size of mitral valve annulus larger, than previously published values.
Cytotoxic effects of cardioplegic solutions and cytoprotective effects of insulin on immature cardiac myocytes during hypothermic preservation
Authors: Orita Hiroyuki , Fukasawa Manabu , Uchino Hideaki , Uchida Tetsuro , Shiono Satoshi , Washio Masahiko
Published: August 2008

The purpose of this study was to evaluate the functional and biochemical effects of cardioplegic solutions on immature cardiac myocytes incubated under hypothermic conditions. In addition, the effects of insulin as an additive were evaluated in each solution. Cardiac myocytes were isolated from neonatal rat ventricles and cultured for four days; 12.5 x 105myocytes/flask were then incubated at 4 °C for three, six and 12 hours in three types of cardioplegic solutions—glucose-potassium solution (glucose: 50 gm/l, NaHCO3: 20 mEq, KCl: 20 mEq), lactated Ringer's solution (KCl: 20 mEq) and St. Thomas' Hospital solution. After each hypothermic incubation, enzymes were measured in the incubation solutions. The myocytes were then cultured for an additional 24 hours at 37 °C to evaluate the recovery of the myocyte beating rate. In the Ringer's group, the recovery ratio of the myocyte beating rate was complete at three hours, then decreased to 48.8 percent of control (beating rate prior to hypothermic incubation) at 12 hours. The glucose-potassium and St. Thomas' groups had significantly lower recovery ratios than the Ringer's group, beginning at three hours (63.4, 72.9, 95.6 percent, respectively). Release of enzymes (CPK and LDH) in the Ringer's group gradually increased and at 12 hours was 29.0 mIU/flask and 260.0 mIU/flask, respectively. The St. Thomas' group, in contrast, had significantly increased values for CPK at 12 hours to 116.0 mIU/flask, and the greatest increases of both enzymes were observed in the glucose-potassium group at 12 hours (CPK: 115.5, LDH: 1163.9). By addition of 20 IU/l insulin, marked improvements were observed in the Ringer's and glucose-potassium groups both functionally and biochemically. Thus, the lactated Ringer's solution had the least cytotoxic effects that might be suitable for a basic solution of various cardioplegic solutions during the neonatal period, and insulin may have beneficial effects on immature myocardium under hypothermic conditions.
Double orifice left atrioventricular valve—diagnosis and management of an unexpected lesion
Authors: Brieger David B. , Ward Cameron , Cooper Stephen G. , Nunn Graham R. , Cartmill Timothy B. , Sholler Gary F.
Published: August 2008

Double orifice left atrioventricular valve is an uncommon condition. This paper describes 10 consecutive patients with the anomaly encountered over 20 months. Diagnosis was assisted by cross-sectional echocardiography and supplementary color Doppler, often requiring non-standard views and a high index of suspicion. There were three anatomical variants—those in which the orifices were equal in size (three of 10 patients), those in which they were unequal and associated with an atrioventricular septal defect (four of 10 patients), and those in which they were unequal in the absence of an atrioventricular septal defect (three of 10 patients). The unequal orifice in both anatomical situations was frequently complicated by valvar regurgitation (six of seven patients). Conservative repair was possible in three of the four patients requiring surgery to the left atrioventricular valve. This condition should be thought of more often in the differential diagnosis of left atrioventricular regurgitation in childhood.
Mycotic aneurysm of the pulmonary trunk in a patient with valvar pulmonic stenosis
Authors: Abbag Fuad , Galal Omar
Published: August 2008

A seven-year-old patient with severe valvar pulmonary stenosis developed a mycotic aneurysm of the pulmonary trunk, which was followed closely with echocardiography. The aneurysm increased rapidly in size over a period of three weeks and required surgical removal.
Long-term cardiac follow-up of children with perinatally acquired human immunodeficiency virus-type 1 infection
Authors: De Simone Luciano , de Martino Maurizio , Galli Luisa , Manetti Adriano , Favilli Silvia , Pollini Iva , Calabri Giuseppe , Mannelli Francesco , Vierucci Alberto
Published: August 2008

Since 1985 a physical and instrumental cardiac study was carried out in 41 children with perinatal HIV-1 infection in order to assess the prevalence and natural history of cardiac involvement. Children were followed for 66 (range 8-109) months; eight of 41 died of HIV-1 related diseases. The cumulative proportion of children with cardiac manifestations at age of seven years was 14.9% (95% CI: 4.9-31.5%). Cardiac abnormalities were detected in eight of 41 patients (19.5%) and were more significantly (p=0.003) recorded in children who died of HIV-1 related diseases (five of eight, 62.5%). Left ventricular systolic function was normal in most patients during the follow-up and did not depend on zidovudine treatment. Two patients had a severe dilated cardiomyopathy and underwent subsequently a stable regression as far as the clinical and instrumental frame was concerned. Only one patient died because of cardiac disease. We conclude that in our group ofperinatally HIV- 1 infected children, cardiac involvement is relatively rare, represents a late complication compared to other related HIV-1 diseases and occurs in advanced stages of illness. Cardiac disease is therefore a hallmark of poor prognosis, but is rarely the ultimate cause of death.
Unguarded tricuspid orifice with Uhl's malformation
Authors: Agarwala Brojendra , Waldman J. Deane , Carbone Michele
Published: August 2008

A case is reported where unguarding of the tricuspid orifice was associated with Uhl's malformation. Echocardiographic features of the condition permitted the diagnosis during life. A therapeutic plan is proposed which involves surgical closure of the unguarded tricuspid orifice.
Muscular ventricular septal defect in a human embryo of 11 mm crown-rump length (six weeks)—no case of developmental arrest
Authors: Wenink Arnold C. G.
Published: August 2008

A human embryo of 11 mm crown-rump length (six weeks, stage 15) is described, in which a muscular defect is present in the ventricular septum, in addition to the interventricular communication normal for this stage of development. The defect consists of large confluent spaces between very loose trabeculations. Its morphology is identical to that of the central muscular ventricular septal defect well recognized in the mature heart. It is suggested that the defect was caused by widening of the sinusoidal spaces which are temporarily present in the normal developing septum.
Hemodynamic and angiographic findings following arterial switch repair for complete transposition
Authors: Srinivas Arun , Ranjit Madathil , Wilkinson James L. , Goh Tiow , Edis Brian , Menahem Samuel , Fong Lance , Weintraub Robert
Published: August 2008

The arterial switch operation is the treatment of choice for complete transposition, and for the Taussig-Bing anomaly, with good early and mid-term results. This retrospective study examined the findings obtained at routine follow-up cardiac catheterization after primary arterial switch repair. We catheterized 111 patients after a mean of 16.9 months after surgery. These included 67 patients with an intact ventricular septum, 33 with a ventricular septal defect, and 11 with the Taussig-Bing anomaly. Right ventricular pressures were mildly elevated (mean 33.9±10.2 mm Hg) in the overall group with a mean pressure ratio between right and left ventricles of 0.34±0.1. Of the patients 74% had gradients across the right ventricular outflow tract of under 20 mm Hg, while three (2.7%) had gradients over 30 mm Hg. A significant gradient across the left ventricular outflow tract occurred in one patient (0.9%), while significant neo-aortic stenosis was not seen. The neo-aortic root remained dilated, with mild aortic valvar incompetence being seen in 12%, with none having higher grades of regurgitation. Left ventricular ejection fraction was normal in all patients, while left ventricular end-diastolic pressure was elevated in 38%. Coronary arterial stenosis was not seen, but one patient (0.9%) had left ventricular apical dyskinesia. Overall, therefore, we conclude that cardiac hemodynamics and ventricular systolic function after primary arterial switch are good. Minor gradients to the pulmonary arteries, and mild neo-aortic valvar incompetence were commonly noted. Left ventricular end-diastolic pressure was elevated in over a third of the patients. These subclinical and subtle abnormalities warrant ongoing follow-up to determine their long-term significance.
Early definitive repair of tetralogy of Fallot: a review of 74 cases
Authors: Abramov Dan , Barak Jacob , Raanani Ehud , Birk Einat , Vidne Bernardo A.
Published: August 2008

Definitive repair of tetralogy of Fallot was performed on 74 patients under two years of age, including 40 under the age of one year and 13 less than 6 months of age. In six patients, there had been previous construction of a systemic-to-pulmonary arterial shunt. Patching across the ventriculo-pulmonary junction was required in 46 patients, placement of conduits in six, and procedures producing unifocaliza-tion in three. Three patients died during the 60-day postoperative period of observation. Of the 71 survivors, 64 recovered without complications. One patient had neurologic sequels, one required prolonged mechanical ventilation, two reoperation, and one balloon dilation of residual left pulmonary arterial stenosis. Morbidity and mortality were not significantly higher in those patients undergoing surgery at earlier than 6 months of age, although transjunctional patching was more common in that age group. Previous construction of a shunt was associated with a higher prevalence of distal stenosis and distortion of the pulmonary arteries, which needed surgical repair in two out of six patients (33%). Since the outcome of definitive repair is favourable in patients with tetralogy of Fallot younger than six months of age, we now advocate definitive surgery for all young symptomatic patients by the age of six months.
Echocardiographic delineation of anomalous origin of the right subclavian artery from the right pulmonary artery
Authors: Law Yuk , Smallhorn Jeff , Adatia Ian
Published: August 2008

We describe a case of anomalous origin of the right subclavian artery from the right pulmonary artery detected by cross-sectional echocardiography in an infant with type B interruption of the aortic arch. Preoperative recognition and surgical reimplantation of the right subclavian artery are important to prevent subsequent subclavian steal syndrome.
Double inlet left ventricle associated with a subpulmonary tissue tag, dissection of the pulmonary trunk and pheochromocytoma
Authors: Medlicott Shaun A.C. , Harder Joyce R. , Denmark Lloyd N.
Published: August 2008

Previous studies have demonstrated the individual association of subpulmonary tissue tags, dissection of the pulmonary trunk and primitive neuroectodermal tumors with congenital heart disease. We are reporting a case of double inlet left ventricle with all these associations as it is exceptionally rare for all features to be manifest in one individual.
Coexistence of atrioventricular block and Wolff-Parkinson-White syndrome in children
Authors: Sigfússon Gunnlaugur , Webber Steven A. , Beerman Lee B.
Published: August 2008

Coexistence of atrioventricular block and Wolff-Parkinson-White syndrome is uncommon, but may occur in children, particularly with certain congenital heart defects. Our study describes 5 children who had pre-excitation and underlying disturbance of atrioventricular conduction. All of these patients had structural heart defects, consisting of discordant atrioventricular connections in three, double inlet left ventricle in one, and a ventricular septal defect associated with straddling of the tricuspid valve in the other. In 4 of the patients, the presence of atrioventricular block was initially masked by the pre-excitation. When the conduction through an accessory pathway is lost, the child may be left with atrioventricular block, which is poorly tolerated in the setting of complex cardiac anatomy. Awareness of this association of Wolff-Parkinson-White syndrome and atrioventricular block is important in understanding the natural history and the management of children with congenital heart defects that are associated with tenuous atrioventricular conduction.
Successful one stage biventricular correction of aortic atresia with a ventricular septal defect and discordant ventriculo-arterial connections
Authors: Ebels Tjark , Dapper Friedhelm , Bauer Jurgen , Bohle Rainer M. , Hagel Karl J. , Nikkels Peter G.J. , Hehrlein Friedrich W.
Published: August 2008

Aortic atresia is rare in the setting of a normally developed left ventricle with a ventricular septal defect. In this combination, as far as we know, it has been described only with concordant ventriculo-arterial connections, for which seven one-stage biventricular repairs have now been described. We describe here the case of a 3½-month-old male infant with a similar combination but with discordant ventriculo-arterial connections and severe pulmonary hypertension. It was the right ventricle which achieved normal size in this arrangement, presumably because of the ventricular septal defect. One-stage biventricular correction was accomplished, employing a single pulmonary allograft, aided by massive doses of Prostaglandin El. As far as we know this is the first report of the combination of aortic atresia, discordant ventriculo-arterial connections, a ventricular septal defect and balanced ventricles. We complement our surgical account, nonetheless, with a description of a comparable specimen from our anatomic museum.
Use of balloon expandable stents in the palliative relief of obstructed right ventricular conduits
Authors: Hayes Alison M. , Nykanen David G. , McCrindle Brian W. , Smallhorn Jeffrey F. , Freedom Robert M. , Benson Lee N.
Published: August 2008

Objectives

The purpose of this study was to determine the efficacy of balloon expandable stents in the relief of obstructed conduits in the right heart, and to review both the immediate success and longevity of this palliation.

Methods and results

Endovascular stents where placed in 17 children, median age 4.7 yrs (range 0.46–16.2 yrs), at a median of 1.6 yrs (range 0.33–6.7 yrs) after placement of conduits at surgery. A second stent was required in two children, either because of migration of the initial stent or residual proximal stenosis. Mean right ventricular to systemic pressure ratios were reduced from 0.77 ±0.24 to 0.50±0.13, and the minimum area of the pulmonary outflow tract increased from 24±12% to 69±26% of that expected for body surface area. Further surgery was needed in 8 patients following placement of the stent, in 5 within 10 months of implantation, due to inadequate relief of obstruction. Nonetheless, 3 of these 8 children achieved satisfac­tory palliation 18, 21 and 24 months prior to requiring insertion of a new conduit. Actuarial freedom from reoperation was 44% at 24 months. The remaining 9 children continue to benefit from the relief in obstruction provided by the stent at a mean interval from implantation of 24±8 months. In this group, the mean Doppler gradient across the right ventricular outflow tract was 35 ±13 mmHg (n=6).

Conclusions

These early data indicate that endovascular stents are useful in the palliation of obstructed right ventricular conduits. In the short-term, the relief of obstruction achieved is well maintained, and has either delayed or obviated the need for insertion of new conduits.
Misleading echocardiographic diagnosis of anomalous origin of the right coronary artery from the pulmonary artery
Authors: Michielon G. , Stellin G. , Milanesi O.
Published: August 2008

We report on a case of a newborn with echocardiographic diagnosis of anomalous origin of the right coronary artery from the main pulmonary artery. Intraoperative inspection demonstrated a proximal anomalous course of the right coronary artery but a normal origin from the right sinus of Vansalva of the Aorta. We suggest a complete investigation, including coronary angiography, when isolated and rare congenitaj malformations of the coronary arteries are suspected.
Surgical correction of a patient with discordant atrioventricular and concordant ventriculo-arterial connections
Authors: Morita Kiyozo , Kurosawa Hiromi , Miyamoto Hisaki
Published: August 2008

The combination of discordant atrioventricular and concordant ventriculoarterial connections is an extremely rare entity, with only 8 cases of successful surgical repair being previously reported. Although from the clinical standpoint this anomaly is similar to complete transposition, this unusual combination is clearly suited by its morpho-physiological features to an atrial rather than an arterial switch procedure. A 17-year-old boy with this malformation, associated also with severe pulmonary hypertension, underwent a palliative Senning procedure, leaving the ventricular septal defect open and banding the pulmonary trunk. Postoperatively, hypoxemia was markedly improved, and was associated with a reduction of the level of pulmonary hypertension, contributing to marked symptomatic improvement.
The echocardiographic anatomy of ventricular septal defects
Authors: Gatzoulis Michael A. , Li Jia , Ho Siew Yen
Published: August 2008

Many of the controversies surrounding the description of ventricular septal defects arise from differences in the perspectives from which they are viewed. In this review, we analyse these defects as seen in cross-sectional echocardiographic images, correlating them with morphologic specimens. The classification we advocate, which now has a suitable pedigree, is a simple descriptive method distinguishing between perimembranous, muscular, and doubly committed types of defects. The approach is to categorise the defects as seen from the right ventricle, the usual port of access for surgeons. The term ‘perimembranous’ highlights the proximity of the atrioventricular conduction axis to the margin of the defects in which the remnant of the membranous septum forms a direct border. This system is applicable to all interventricular communications, no matter how malformed the heart may be in which they are enclosed.
Effects of positive pressure ventilation and inspired oxygen on pulmonary vascular resistance and tissue oxygen delivery in neonatal pigs
Authors: Ransbæk Flemming , Hansen Soren B , Austin Erle H. , Santamore William P.
Published: August 2008

Management of pulmonary vascular resistance in neonates with congenital heart disease is important for stabilization before and after surgical interventions. Thus, we determined which combina tion of positive end-expiratory pressure ventilation and fraction of oxygen in the inspired air increases pulmonary vascular resistance without compromising delivery of oxygen to the tissue. Eight piglets were anesthetized, intubated and ventilated. Pulmonary flow and pulmonary arterial and left atrial pressures were monitored continuously. At all levels of inspired oxygen (1.00, 0.21 and 0.15), ventilation at a pressure of 15 cm of water increased pulmonary vascular resistance. At all levels of positive pressure ventilation, a fraction of 0.15 of inspired oxygen increased pulmonary vascular resistance. The combination of a ventilatory pressure of 15 cm of water and inspired oxygen of 1.00, or ventilatory pressure at 5 cm of water and oxygen delivery of 0.15, produced similar changes in pulmonary vascular resistance (19.1 ± 2.8 vs. 20.0 ± 3.8 mmHg/(L/min)) and cardiac output (0.78 ± 0.07 vs. 0.93 ± 0.10 L/min) but, the higher level of positive pressure plus 1.00 inspired oxygen gave a significantly higher arterial oxygen saturation (0.99 ± 0.03 vs. 0.72 ± 0.19%) and delivery of oxygen to the tissues (13.7 ± 2.9 vs. 7.4 ± 1.5 ml 02/min, p<0.05). Thus, both high positive pressure ventilation and hypoxia increase pulmonary vascular resistance. Only high pressure ventilation plus high concentrations of inspired oxygen, however, increased pulmonary vascular resistance without compromising delivery of oxygen, suggesting that this combination is a superior means of increasing pulmonary vascular resistance.
Palliative arterial switch for complete transposition with ventricular septal defect
Authors: Elizari Amalia , Somerville Jane
Published: August 2008

A 28-year-old female patient with complete transposition, ventricular septal defect and persistence of the arterial duct underwent a palliative arterial switch procedure in 1976 at 7 years of age. Therefore, she has survived for 22 years and lives a near normal life. She is married, has been counselled against pregnancy and has increasing cyanosis with the typical features of the Eisenmenger syndrome.
‘You didn't see them, but now you do!': use of balloon occlusion angiography in the identification of systemic venous anomalies before and after cavopulmonary procedures
Authors: Ovaert Caroline , Filippini Luc H. P. M , Benson Lee M. , Freedom Robert M.
Published: August 2008

We describe the importance of angiographic identification of collateral venous channels by balloon occlusion venography after bidirectional cavopulmonary connections. Use of the balloon occlusion technique is essential for identification of these vessels, as they can easily be missed by standard venous angiography, with possible clinical consequences for postoperative management.
Successful management of a pregnancy at high risk because of Eisenmenger reaction
Authors: Daliento Luciano , Menti Liana , Lenardo Laura Di
Published: August 2008

Informed medical care, appropriate maternal and fetal monitoring, and cooperation among experienced obstetricians, anesthesists cardiologists, particularly during delivery and the early puerperium associated with psychological preparation of the mother, were the bases of the successful management of a pregnant 28-year-old woman at high risk because of the Eisenmenger reaction.
Atrial thrombus after the Fontan-operation: predisposing factors, treatment and prophylaxis
Authors: Kaulitz R. , Ziemer G. , Bergmann F. , Luhmer I. , Kallfelz H. C.
Published: August 2008

Thrombosis of the right side of the heart has previously been a rarely reported early or late postoperetive complication after a modified Fontan-procedure.

In 4(5.5%) of 72 surviving patients who underwent a total cavopulmonary connection for various forms of univentricular atrioventricular connection or other complex lesion a thrombus within the right atrium was diagnosed by transthoracic echocardiogaphy either early (in 3 cases) or late (in one cases) postoperatively. Predisposing haemodynamic abnormalities could be identified in all four patients, but no patient was found to have severe abnormalities of coagulation. Two patients presented with acute clinical symptoms on the third and twelfth day after initial surgery. They underwent emergency reoperation with thrombectomy followed by prophylactic treatment with coumadin or aspirin after initial heparinization. In the other 2 patients, routine postoperative echocardiography revealed a right atrial thrombus without specific clinical symptoms 25 days or 3 months postoperatively. Thrombolytic therapy with an initial bolus of tissue plasminogen activator (0.1 mg/kg) followed by continuous infusion (1 mg/kg/day) combined with heparinization yielded complete resolution of the thrombus after 27 and 40 hours, respectively, as demonstrated on follow-up echocardiograms. Our own experience, coupled with a review of the literature, indicate that prophylactic anticoagulation therapy with coumadin should continue for at least 4 to 6 months for patients with an increased risk for postoperative thrombosis. For infants and young children with a low risk for postoperative thrombosis, aspirin should be given routinely for 6 months postoperatively.
Morphometric study of the great arterial trunks and their branches in the human fetal heart with perimembranous ventricular septal defects
Authors: Alvarez Luis , Aránega Antonia , Vélez Celia , Melguizo Consolación , González Amanda R. , Saucedo Robert
Published: August 2008

Morphometric methods were used to study the great arterial trunks in a total of 22 human fetuses and newborn subjects weighing from 1 to 3.8 kg (mean 2.22 ± 0.85 kg). All specimens displayed concordant atrioventricular and ventriculoarterial connections, and all had perimembranous ventricular septal defects. Thirteen different parameters were measured in each specimen, comprising the orifices of the great arterial trunks, ascending aorta, vessels branching from the aortic arch, the aortic isthmus, descending aorta, pulmonary trunk, orifices of the pulmonary arteries, and arterial duct. The values were Compared with patterns of normality established in an earlier study using hearts from 496 human fetuses and neonates weighing form 60 to 5000 g. Minimum square regression analyses were used to study the relationships between fetal and neonatal log body weight in kilograms, and the various cardiometric parameters. We believe that these morphometric data have immediate clinical and surgical applications in the treatment of fetal and neonatal cardiovascular disorders.
The feasibility of high-dose dobutamine stress echocardiography in children
Authors: Pahl Elfriede , Chrystof Dale , Webb Catherine L. , Shulman Stanford T. , Chaudhry Farooq A.
Published: August 2008

We recently reported our experience with exercise stress echocardiography in children with prior history of Kawasaki disease and coronary arterial involvement. Dobutamine stress echocardiography has been used in adults (doses up to 50 μg/kg/min) for diagnosis and risk stratification of coronary arterial disease. To date, no studies have assessed the safety and utility of high-dose dobutamine for the detection of abnormalities of wall motion in children, although low dose dobutamine (5 μg/kg/min) has been used to assess left ventricular function after use of anthracycline in children. Our study group consisted of 10 children known to have Kawasaki disease, and 16 children who had undergone heart transplantation. After baseline transthoracic echocardiographic imaging, dobutamine was infused in an incremental fashion, 10–40 μg/kg/min intravenously, to attain peak heart rate. Heart rate and blood pressure were monitored continuously, and 4 standard echocardiographic images were acquired at each stage of dobutamine infusion and at recovery. Images were digitized, and the left ventricular segments were reviewed to assess wall motion. No patients suffered serious side effects such as arrhythmias or hypotension. Studies were complete in all 26 patients. One study proved positive, with a new abnormality of wall motion identified corresponding to a critical right coronary arterial stenosis. Peak heart rate and blood pressure were similar for the 2 groups. High dose dobutamine stress echocardiography, therefore, is feasible and safe in children, and may detect flow-limiting coronary arterial stenoses noninvasively. The technique may safely be applied to other subsets of children where assessment of ischemia and contractile reserve is warranted.
Post-Operative cardiac strangulation
Authors: Waldman J.Deane , Karp Robert B. , Ferdinand Francis
Published: August 2008

A patient is reported in whom cardiac strangulation occurred through a lateral pericardial defect surgically created for repair of congenital heart disease. To avoid this potentially fatal complication, the inferior rim of an iatrogenic lateral pericardial defect should be tacked down to preclude cardiac herniation.
A possible role for steroid therapy in preventing postoperative stenosis in totally anomalous pulmonary venous connection
Authors: Sands Andrew , Craig Brian , Casey Frank
Published: August 2008

We report three cases of pulmonary venous stenosis developing after repair of totally anomalous pulmonary venous connection. In each case, we used steroids as a therapeutic measure following re-operation. In two cases, we were successful in preventing significant re-stenosis. The third patient rapidly developed stenosis (mean pulmonary wedge pressure approximately 20 mmHg at cardiac catheterisation) after stopping steroids. This patient subsequently died after a third operation. The remaining patients continue to do well.
Persistence of the hepatic venous plexus with underdevelopment of the inferior caval vein: implications in the management of complex congenital heart disease
Authors: Gladman Gordon , Adatia Ian , Freedom Robert M.
Published: August 2008

We report two cases with persistence of the hepatic venous complex, underdevelopment of the inferior caval vein and azygos continuation to the superior caval vein. We suggest that the hepatic venous complex is a true congenital malformation rather than the result of passive enlargement of venous channels subsequent to high venous pressures. Preoperative diagnosis is important in patients with complex congenital heart disease undergoing palliative venous redirection procedures if a postoperative right-to-left shunt and cyanosis are to be avoided.
Disparity between the arrangement of the abdominal vessels and the bronchial tree and atrial appendages in a patient with mirror-imaged venous return
Authors: Soukias Nikos D. , Shinebourne Elliot A. , Carvalho Julene S.
Published: August 2008

Either arrangement of the abdominal vessels or the morphology of the bronchial tree are usually used to predict atrial arrangement. We report a patient in whom, in the setting of normal physiology as seen with mirror-imaged venous return, and due to disparity between the arrangement of the abdominal vessels (suggestive of right isomerism) and thoracic arrangement (indicative for left isomerism), atrial arrangement could not be inferred. Direct echocardiographic imaging of both appendages revealed left isomerism.
Mitral valvar prolapse and regurgitation combined with aortic regurgitation in a child with Sanfilippo syndrome type A
Authors: Alturjuman Ahmad , Mehta Ashok V.
Published: August 2008

Cardiovascular involvement is commonly reported in various muco- polysaccharidoses. We report a first case of Sanfilippo syndrome type A in a 12-year-old white female who has developed combined progressive mitral valvar regurgitation due to prolapse and aortic regurgitation.
Complete surgical resection of intrapericardial teratoma in a neonate with compression of the central airways
Authors: Sievers Roy F. B. , Tang Augustine T. M. , Haw Marcus P.
Published: August 2008

Intrapericardial teratoma is a rare but recognised cause of respiratory distress in neonates. Patients often present with the compressive effects of the mass within the thorax. Prompt diagnosis should be followed swiftly by surgical resection. We report an unusual case of intrapericardial teratoma in a neonate presenting with collapse of the lung which was successfully treated by surgery.