bluuebunny/crossref_metadata_2025 huggingface dataset:
Magnetic resonance in the detection and review of aortic aneurysms following surgical repair of aortic coarctation
Authors: Hayes Alison M. , Baker Edwards J. , Murdoch Ian , Rigby Michael L.
Published: August 2008

Formation of aortic aneurysms is an important and not infrequent sequel of surgical repair of aortic coarctation. Detection is often haphazard and fatal ruptures are reported. We report a series of cases where resonance imaging identified aneurysmal dilatation of the thoracic aorta after either patch aortoplasry or subclavian flap repair of aortic coarctation. In one, rupture of the aneurysm occurred five months following the initial resonance study. Repeat imaging confirmed the clinical suspicion of rupture and successful surgical resection was subsequently performed. Magnetic resonance is a practical and reproducible imaging technique which is particularly valuable in the identification of abnormalities within the thoracic aorta following surgical aortoplasty or interventional procedures for aortic coarctation. As a method of routine surveillance in these groups of patients, it is superior to any alternative technique.
Non-cardiac cyanosis in left isomerism—report of two cases of multiple pulmonary arteriovenous malformations
Authors: Burch Michael , Iacovides Paris , Habibi Parviz , Celermajer David
Published: August 2008

Isomerism of the left atrial appendages is often associated with cyanosis due to intracardiac structural abnormalities such as common atrium and/or anomalous pulmonary or systemic venous connections. We report two cases of children with cyanosis and left isomerism but no other lesions within the heart. In these cases, multiple small pulmonary arteriovenous malformations were found bilaterally, resulting in intrapulmonary right-to-left shunting and systemic desaturation.
Two-stage repair in a patient with “absent pulmonary valve syndrome”
Authors: Tamimi Hussam , Galal Omar , Halees Zohair Al
Published: August 2008

A seven-month-old boy with severe respiratory distress because of absence of the leaflets of the pulmonary valve underwent banding of the pulmonary trunk. Postoperative recovery was uneventful with dramatic improvement of his general and hemodynamic status. Repair was performed successfully almost two years later when the child had grown satisfactorily and was in stable condition.
Appearance of a localized bulge in the anterior aspect of the descending aorta after balloon angioplasty of native aortic coarctation—aneurysm or reopening of the arterial duct?
Authors: Galal Omar , Vogel Michael , Bjoernstad Per
Published: August 2008

Balloon dilation of native aortic coarctation was performed in a three-month-old boy. Angiography immediately after dilation revealed a localized bulge on the anterior as pect of the descending aorta interpreted initially as an aneurysm. Angiography four months later, however, failed to detect this bulge. We speculate that the early aneurysmal appearance of the anterior wall of the aorta may have been caused by factors other than a tear in the aortic adventitia.
Insertion of a bypass graft from carotid artery to descending aorta in the management of aortic coarctation with severe hypoplasia of the aortic arch—a report of two cases
Authors: Prabhakar Ganga , Kumar Naresh , Halees Zohair Al , Wilson Neil
Published: August 2008

Repair of severe hypoplasia of the aortic arch with coarctation must be based on the individual anatomy of the lesion and, where necessary, one should take into consideration associated cardiac abnormalities. We report a surgical technique which was employed when standard procedures for reconstruction of the arch had failed to relieve the obstruction adequately. A conduit from the carotid artery to the descending aorta was used successfully in two patients to abolish residual stenosis.
Malnutrition and severe rheumatic heart disease underscoring failure to thrive in an eleven-year-old boy
Authors: Saraiva Lurildo Ribeiro , Barbalho Tacito , Filho Djair Brindeiro
Published: August 2008

We describe an eleven-year-old boy with severe retardation in physical growth and severe rheumatic valvar disease. At surgery, a sample of the atrial appendage showed Aschoff nodules, although no antecedent of rheumatic fever was recorded. The patient was HLA-DR5 positive. The case emphasizes the strong association of malnutrition with severe rheumatic heart disease in northeast Brazil.
Subpulmonary tag originating from both sides of the ventricular septum in congenitally corrected transposition
Authors: O'Sullivan John J. , Farrell Desmond J. , Hunter Archibald S.
Published: August 2008

Obstruction to the left ventricular outflow tract is not uncommon in patients with congenitally corrected transposition. A case of congenitally corrected transposition is described with significant subpulmonary obstruction which was due to a subpulmonary tag. The patient required an aortopulmonary shunt. Her clinical course was subsequently significantly influenced by arrhythmias associated with an accessory pathway. She died suddenly from an arrhythmia. Post-mortem examination confirmed a large subpulmonary tag which originated from both sides of the ventricular septum—a previously unreported finding. Careful evaluation of the nature of left ventricular outflow obstruction is important in this condition as it may alter the surgical management.
Totally anomalous pulmonary venous connection through the right lung and via a “Scimitar” vein to the inferior caval vein
Authors: Dua Rakesh , McTigue Christine , Wilkinson James.L
Published: August 2008

We report a case of totally anomalous pulmonary venous connection in which the two pulmonary veins from the left lung joined to form a common vein which then passed across the midline into a hypoplastic right lung and, after receiving small veins from the right lung, passed inferiorly, exiting the lung below the hilum as a “scimitar” vein and terminating in the inferior caval vein. A separate pulmonary vein from the right lung passed inferiorly independently and joined the “scimitar” vein before it entered the inferior caval vein. There was an associated hypoplastic left heart syndrome.
The value of resonance imaging in diagnosis of pulmonary mycotic aneurysm secondary to infective endocarditis
Authors: Zabal Carlos , Ramírez-Marroqín Samuel , Attie Fause
Published: August 2008

Mycotic aneurysms of the pulmonary trunk are a rare but recognized complication of infective endocarditis. They are usually associated with infected congenital heart lesions, and especially with persistent patency of the arterial duct. This report deals with a patient with a persistent duct and aortic coarctation who developed a mycotic aneurysm of the pulmonary trunk secondary to infective endocarditis. The lesion was evaluated before and after surgery by magnetic resonance imaging.
An angiographic study of coronary arterial disease in children after heart transplantation
Authors: Ladeia Ana , Bidois Jérôme Le , Piéchaud Jean François , Metzger Jean Philippe , Vouhé Pascal , Kachaner Jean
Published: August 2008

Coronary angiograms were analyzed in 29 children after heart transplantation. Age at transplantation was one month to 14 years (mean 4.5 years) with follow-up from five months to six years (mean 2.9 years). Immunosuppression was based on cyclosporine and azathioprine; six patients also received maintenance steroids. Coronary angiograms were obtained after giving an intracoronary arterial bolus of nitroglycerin to avoid spasm. Coronary angiograms showed stenoses, luminal irregularities, loss of luminal diameter and obliteration of branches, and one example of myocardial bridging. The review of angiographic recordings in some patients showed a loss of normal motion of coronary arteries which, instead, appeared rigid. No abnormalities were seen in eight patients (28%). Coronary arterial changes were present between six months and four years after transplantation in 15 patients (52%). Isolated loss of normal motion of the coronary arteries was present in six patients (20%). The separate analysis of 15 patients who underwent more than one coronary angiogram showed a progression of lesions in eight cases. Coronary arterial disease is a frequent complication of heart transplantation in children and there is a progression of lesions in a large proportion of patients. Since there is no satisfactory therapy for this complication, heart transplantation should be reserved for children having no other reasonable therapeutic option.
Embryonic ventricular diastolic and systolic pressure-volume relations
Authors: Keller Bradley B. , Tinney Joseph P. , Hu Norman
Published: August 2008

The embryonic heart and vascular bed interact dynamically to support rapid growth of the embryo during cardiovascular development. Pressure-volume relations define ventricular function during alterations in loading conditions. We analyzed these relationships in the embryonic heart in order to define ventricular function and the response of the ventricle and vascular bed to acute changes in preload. We simultaneously measured ventricular pressure and recorded 60 video images per second in n≥6 stage 16, 18 and 21 white Leghorn chick embryos at baseline and during the infusion of 1–2 microliters of physiologic buffer into the venous sinus (sinus venosus). Ventricular tetany was then induced with the topical application of 2 Molar sodium chloride. Video fields were traced for ventricular pressure and epicardial cross-sectional area. Cross-sectional area was converted to volume assuming ellipsoid geometry, and cavity volume was calculated as total volume minus wall volume derived from the tetanized heart. We defined end-diastole at the onset of ventricular contraction and end-systole at maximum pressure/volume ratio. Stroke volume increased linearly with end-diastolic volume. End-diastolic pressure-volume relations were positive and linear, and end-systolic pressure-volume relations were curvilinear downward. Arterial elastance decreased with growth of the embryo and with volume infusion. Pressure-volume loop area, an index of consumption of energy, doubled between the embryonic stages. Thus, embryonic ventricular pressure-volume relations define diastolic and systolic function at rest and in response to altered preload.
Catheter ablation in children with supraventricular tachycardia mediated by accessory pathways—use of radiofrequency current as a first line of therapy
Authors: Hebe Joachim , Schlüter Michael , Kuck Karl-Heinz
Published: August 2008

Catheter ablation of an accessory atrioventricular pathway using 500 kHz radiofrequency current was attempted in 53 children and young adolescents less than 16 years of age (mean 9.3±4.1 years) who were referred for treatment of symptomatic supraventricular tachycardia. Thirty children had the Wolff-Parkinson-White syndrome and 23 had tachyarrhythmias related to an accessory pathway conducting only in retrograde fashion. Of the latter, seven were found to have the permanent form of junctional reciprocating tachycardia, which had resulted in depressed left ventricular function in four. Ablation of left-sided accessory pathways was usually attempted utilizing an arterial approach to the annulus of the mitral valve, while the venous route to the atrial aspect of the tricuspid valvar annulus was chosen for right-sided accessory connections. To reduce invasiveness and the duration of the procedure, a technique with a solitary catheter was attempted in 20 children with the Wolff-Parkinson-White syndrome, regardless of the anatomical site of the accessory pathway. Ablation of 54 of 56 accessory connections was achieved in 51 patients (96% success) with a median of six applications of radiofrequency current. The sessions were completed within 3.4±2.2 hours, with a median exposure to radiation of 31.1 minutes. During a 10-month period of follow- up, no patient required antiarrhythmic medication, and 51 children (96%) were completely free of any symptoms related to arrhythmias. Left ventricular function returned to normal in the four children with tachycardia-induced cardiomyopathy. Except for a patient with an arterial thrombotic occlusion, no serious complications were encountered. Catheter ablation using radiofrequency current is a highly effective and safe curative approach for treating young patients with supraventricular tachycardias mediated by accessory pathways. The use of fewer catheters of smaller size is feasible and advisable in this cohort of patients.
An immunohistochemical study of arterial lesions due to pulmonary hypertension in patients with congenital heart defects
Authors: Aiello Vera Demarchi , Lourdes Higuchi Maria de , Lopes Edgard Augusto , Barbosa Lopes Antonio Augusto , Barbero-Marcial Miguel , Ebaid Munir
Published: August 2008

In order to understand some of the cellular mechanisms of interaction in secondary pulmonary vaso-occlusive disease, we studied 21 lung biopsies from patients with different types of congenital cardiac defects. Their ages ranged from four to 248 months (mean 71.5 months; median 41 months). Changes in the cytoskeleton and extracellular matrix were assessed in the arterial wall. Immunostaining was applied to formalin-fixed, paraffin- embedded tissue, using antibodies to muscle-specific actin, vimentin and fibronectin in supra-optimal dilution. The staining for muscle-specific actin in the medial layer revealed a heterogenous pattern, with areas exhibiting low or absent labelling, reflecting a process of dedifferentiation of the smooth muscle cells in those segments. Within intimal proliferative lesions, the expression of muscle-specific actin was variable, being weak in some lesions and strong in those showing concentrically arranged intimal smooth muscle cells, suggesting a reversion of the migrated cells to the contractile phenotype. The endothelial cells of arteries from cases presenting severe qualitative lesions exhibited strong expression of vimentin, reflecting their heightened regenerative activity and/or their necessity to maintain their shape. The expression of fibronectin was greater in the predominantly cellular lesions of the intima when compared to the fibrotic lesions, indicating the role of that matrix glycoprotein in cellular migration and in replicative processes.
Assessment of ventricular septal defect with aortic valvar prolapse by means of echocardiography and angiography
Authors: Wang Jou-Kou , Lue Hung-Chi , Wu Mei-Hwan , Young Ming-Lon , Chiu Ing-Sh , Chang Chung-I , Hou Shou-Hsien , Chu Shu-Hsun , Hung Chi-Ren
Published: August 2008

A total of 80 patients, diagnosed by echocardiography as having ventricular septal defect with aortic valvar prolapse, underwent cardiac catheterization and surgery. Echocardiographic and angiographic results were compared with surgical findings. The ventricular septal defects as observed during surgery were found to be doubly committed and subarterial in 49 (61%), muscular outlet in 10 (13%), and perimembranous in 21(26%). The location had been erroneously categorized by echocardiography and angiography in 12 (15%) and in 15 (19%) patients, respectively. Prolapse of the right coronary leaflet of the aortic valve, as documented by echocardiography, was confirmed by angiography in all but two cases. Prolapse of the noncoronary leaflet was detected by both imaging modalities in three patients. Prolapse of the right coronary and noncoronary leaflets was observed at surgery in 49 and three patients, respectively. The mean size of the ventricular septal defect, when measured by echocardiography, was significantly smaller than that found following surgical measurements (3.3±1.3 vs 8.4±3.8 mm, p<0.001). Our study showed that the ventricular septal defect was erroneously classified in the presence of prolapse of the aortic valve in 15% and 19% of our cases by echocardiography and angiography, respectively. The herniated sinus of Valsalva forming the “roof” of the ventricular septal defect probably redirected the jet across the defect to cause the errors in interpretation. Echocardiography, nevertheless, is as reliable as angiography in our hands in the follow-up of patients with ventricular septal defect opening to the outlet of the right ventricle.
Mitral valvar stenosis in children maintained on hemodialysis for chronic renal failure-clinical significance and an attempt at percutaneous balloon mitral valvoplasty
Authors: Nakazawa Makoto , Takeda Yumiko , Satomi Gengi , Matsumoto Yasutoshi , Kawaguchi Hiroshi , Momma Kazuo , Ito Katsumi , Ando Masahiko
Published: August 2008

We describe three children with chronic renal failure who developed mitral obstructive disease over a period when maintained on hemodialysis. The development and progression of the mitral disease was documented by cardiac catheterization in one child and by Doppler echocardiography in the other two children. These three patients had the lowest body weight among the 16 children undergoing chronic dialysis who were examined cardiologically in our hospital. Autopsy in one child who died from heart failure revealed marked thickening of the leaflets and subvalvar apparatus of the mitral valve with severe obliteration of the intercordal spaces. We found no sign of calcification at the mitral annulus nor at the attachments of the leaflets of the aortic valve, and no Ashoff bodies. We attempted percutaneous catheter balloon valvoplasty in one child which permitted successful renal transplantation after failure of an initial transplantation because of severe pulmonary venous congestion. We conclude that mitral obstructive disease could be a complication of long-term hemodialysis for chronic renal failure. Catheter intervention may serve to release the obstruction, although the procedure is not curative.
Spectrum of valvar abnormalities in Noonan's syndrome—a pathologic study
Authors: Sreeram Narayanswami , Kitchiner Denise , Smith Audrey
Published: August 2008

Four patients with the phenotypic characteristics of Noonan's syndrome presented in infancy with a suspected cardiac defect. The cardiac segmental connections were normal. All four were diagnosed to have valvar pulmonary stenosis by cross-sectional echocardiography, the valve being typically thickened and dysplastic as expected for the syndrome. In addition, all had hypertrophic cardiomyopathy and evidence for important abnormalities of the other cardiac valves. Two of the four patients died following cardiac surgery. Of the other two, one child died during interventional catheterization and another died while awaiting cardiac surgery. The cardiac abnormalities, confirmed at postmortem, were dysplastic pulmonary valve with stenosis (four patients), dysplastic aortic valve (three patients), supravalvar aortic stenosis (one patient), small and dysplastic tricuspid and mitral valves (four patients), hypertrophic obstructive cardiomyopathy affecting both ventricles, with asymmetric septal hypertrophy (four patients), atrial septal defect within the oval fossa (one patient). In addition to the usually described abnormalities of the pulmonary valve and ventricular myocardium, the patients described showed a wide spectrum of valvar dysplasia which has received little attention in the past. If these patients do indeed have Noonan's syndrome, and the evidence supports this contention, then these findings support the hypothesis of a more generalized disorder in the development of the ventricles and valves in the syndrome, which has a variable expression. They also stress the need to look carefully for abnormalities of valves other than the pulmonary valve in patients who present with symptoms in infanscy.
Solitary arterial trunk
Authors: Penny Daniel J. , Dua Rakesh , Wilkinson James L.
Published: August 2008

A heart is described in which there was a solitary arterial trunk that exited from the heart astride a large perimembranous ventricular septal defect, with the pulmonary arterial supply originating entirely from collaterals from the descending aorta. There was no evidence of either hilar pulmonary arteries or an atretic pulmonary trunk. The morphology of the ventricular outlet component, with absence of the outlet septum supports the description of this heart as solitary arterial trunk, rather than tetralogy of Fallot with pulmonary atresia.
Complete heart block as a cause of syncope in hypertrocphic cardiomyopathy
Authors: Calderón-Colmenero Juan , Baltazares Mario , Buendía Alfonso
Published: August 2008

We report a 12-year-old boy with hypertrophic cardiomyopathy presenting with syncope. His electrocardiogram showed first-degree atrioventricular block, complete right bundle branch block, conduction delay in the anterior fascicle of the left bundle branch, and intermittent complete atrioventricular block. We detected hypertrophic cardiomyopathy in the mother. The relationship between the diseases is discussed, and interpreted in the light of previous descriptions.
A simple method of sternal traction to relieve compression of the heart after open heart surgery
Authors: Matsuki Osamu , Yagihara Toshikatsu , Kawashima Yasunaru
Published: August 2008

A simple technique is described with which to relieve postoperative compression of the heart. Although delayed sternal closure has conventionally been used to deal with postoperative compression, this maneuver always exposes the patient to the risk of infection. We have recently employed a simple method of sternal traction when there are signs of compression on temporary sternal closure. One or two couples of sutures in the sternum are used supported by bags of saline. This simple and effective method is applicable to patients, but especially to infants and neonates, without worrying about overindications.
The role of echocardiography in the diagnosis of aorto-left ventricular tunnel
Authors: Kosutic Jovan
Published: August 2008

A case is presented of a 3.5-year-old boy with aorto-left ventricular tunnel. A diagnosis of isolated aortic regurgitation had been made at the age of 11 months following cardiac catheterization with selective left ventricular angiography and aortography. The correct diagnosis was established 2.5 years later following cross-sectional echocardiographic examination combined with color Doppler investigation.
Aneurysm of the arterial duct—a case report and review of the literature
Authors: Amato Joseph J. , Cardarelli Marcelo G. , Bierman Frederick Z.
Published: August 2008

Aneurysmal dilatation is a rare malformation of the arterial duct. Successful repair of this anomaly was achieved in a five-day-old neonate, using deep hypothermia and circulatory arrest. Of the cases of aneurysm of the arterial duct reported in the English literature, only seven have been treated surgically with success.
Perspectives in fetal echocardiography
Authors: Sahn David J.
Published: August 2008

Editor's Note: This discussion is extracted from a lecture and has been minimally edited to maintain the style which we think maintains the spirit as well as the educational content. It is primarily included to offer trainees an introduction to the history and physiology encountered in this evolving specialty. We invite your comments.
Reflections on the past, present and future of pediatric cardiology
Authors: Hoffman Julien I. E.
Published: August 2008

About 1.5 million children are born each year world-wide with congenital heart disease. The nine most frequently occurring lesions are patency of the arterial duct (ductus arteriosus), atrial septal defect within the oval fossa, atrioventricular septal defect, ventricular septal defect, coarctation of the aorta, valvar pulmonary stenosis, valvar aortic stenosis, tetralogy of Fallot, and complete transposition. Their natural history is presented and compared with representative results of surgical treatment. Surgery has markedly improved the outcome for all these lesions, although almost no patients have been followed postoperatively for more than 25 years. Because congenital heart lesions are likely to consume an increasing proportion of cardiological resources in future years, the long-term future in this field rests with prevention of these lesions if at all possible. Recent advances in molecular and developmental biology, some of which are discussed, will certainly aid in diagnosis and may well bring the time at which prevention can be achieved closer to reality.
Provision of care for congenital heart disease in the United Kingdom
Authors: Hunter Stewart
Published: August 2008

The organization of the national health service in the United Kingdom has been under constant review and revision since its inception after the Second World War. Central government spends £35,894 million each year on the Health Service and it is the country's largest employer. Total health expenditure in the United Kingdom accounts for 6.1% of the Gross Domestic Product. This apparently compares unfavorably with 12.4% total health expenditure in the United States.States. However the public health expenditure is identical in the two countries, 5.2%, and this figure is similar in most developed countries including Australia, Denmark, Spain and Switzerland. The overall regional administration of the Health Service in England (Scotland, Wales and Northern Ireland have slightly different organizations) is well established, although the names of the different strata of administration change from time to time. The Secretary of State for Health oversees the Department of Health including the National Health Service Management Executive. Under this Management Executive are 14 Regional Health Authorities covering 100% of the total population.There are within the London area several Special Health Authorities set up in the past because of the particular expertise which they provided. These are under review and will almost certainly lose their special status in the future because of the improvements and increase in services nationwide. The Regional Health Authorities have under their care 177 District Health Authorities and 90 Family Health Service Authorities.They do not manage the National Health Service Trusts—a new development which allows hospitals and other organizations within the Health Service to work autonomously to provide NHS services.
Past, present and future of pediatric cardiology health care in México
Authors: Attie Fause , Cardenas Manuel
Published: August 2008

The Constitution of the United States of Mexico, in its Article Number 4, provides for the right to health care. The government's National Health Program was created to comply with this mandate. This program comprises a National Health Care System. This system has three branches. The first one is Social Security, which encompasses 50% of the population of Mexico—namely 45 million affiliates. Out of this popu lation, 10 million are under the “Instituto Mexicano del Seguro Social-Solidaridad.”This program is financed by federal resources, is self-managed by the “Instituto Mexicano del Seguro Social” and provides health care to the general population (basically farmers). The rest of the population that receives benefits from the Social Security System includes employees, blue-collar workers (affiliated with the “Instituto Mexicano del Seguro Social“), federal and state-government workers (who receive benefit from “Instituto de Seguridad Social de los Trabajadores al Servicio del Estado”) and the military personnel (who are affiliated with the “Instituto de Seguridad Social de las Fuerzas Armadas”). These institutes are jointly financed by employers, the state, and the workers.The payment percentage is established depending on the worker's income. For state workers and armed forces it represents 2% of the income of the employee and another 2% is paid by the state; those depending on die Instituto Mexicano del Seguro Social pay 3.5% of their salary and 8.4% is paid by die employer.
Clinicopathological correlation—a case of complex congenitally corrected transposition
Authors: Schenk Marty , Gerlis Leon M. , Somerville Jane
Published: August 2008

A 29-year-old man, with a diagnosis of congenitally corrected transposition (discordant atrioventricular and ventriculoarterial connections) with associated multiple ventricular septal defects, mild pulmonary valvar stenosis and Ebstein's malformation of a stenotic left-sided morphologically tricuspid valve, died after closure of the ventricular septal defect and insertion of a valved homograft conduit between the morphologically left ventricle and the pulmonary trunk. Death occurred because of failure to appreciate the bizarre and rather stenotic anomaly of the morphologically tricuspid valve, in which the anterosuperior leaflet divided the morphologically right ventricle into an inlet portion and a smaller outlet portion. After closure of the ventricular septal defects, this ventricle could no longer support the systemic circulation. We discuss the natural history and the reasons for the erroneous interpretation of the echocardiographic images of the left-sided tricuspid valve.
Infectious endocarditis—an experience spanning three decades
Authors: Zuberbuhler James R. , Neches William H. , Park Sang C.
Published: August 2008

Summer

Over the last 30 years, 94 episodes of infectious endocarditis have been identified in 86 children seen at the Children's Hospital of Pittsburgh. In most of these children no precipitating event could be documented. There was no change in underlying cardiac anomalies over the three decades of this study, and left ventricular outflow tract obstruction, ventricular septal defect and tetralogy of Fallot were the most common anomalies. Streptococci and staphylococci were the most common infecting organisms, and there was a slight increase in the percentage of streptococci in the last decade. Cardiovascular damage occurred in about one-third of the cases, and aortic regurgitation was most common. Mitral regurgitation was also an important complication, as was the development of an aortic aneurysm. Important systemic and pulmonary emboli were rare. Surgical intervention was necessary in one-fifth of the children. Valvar replacement was the most common procedure done. The mortality rate declined markedly over the period of the study, dropping from 36% in the first decade to 8% in the last.
Efficacy and safety of balloon dilation as palliative treatment for tetralogy of Fallot
Authors: Sharland Gurleen K. , Qureshi Shakeel A. , Ladusans Edmund J. , Parsons Jonathon M. , Baker Edward J. , Deverall Philip B. , Tynan Michael
Published: August 2008

Twenty-six patients requiring palliation for the tetralogy of Fallot were treated by balloon dilation. The mean age at dilation was 8.5 months (range 0.5−20.6) and the mean weight 6.7 kg (range 2.6−10.7). Successful dilation was achieved in 25 patients. The mean ratio of the balloon to the diameter of the pulmonary outflow tract was 1.6 (range 1.2−2.0). The aortic oxygen saturation increased from mean±SD of 80±12.4% before to 91±4.3% after dilation (p<0.05). The pulmonary arterial oxygen saturation rose from 64±10.7% to 80±7.7% (p<0.05). Transient hypotension and arterial desaturation were noted in 19 patients during inflation of the balloon. One patient had a cardiac arrest prior to dilation and died afterwards. Seven patients had cyanotic spells related to the procedure. Attempts to cross the pulmonary valve resulted in infundibular perforation by the guide wire in one patient. Of the 24 survivors who initially had successful dilation procedures, three have needed a Blalock-Taussig shunt. Palliation without a shunt was achieved in the remaining 21 patients. In 12 patients, repeat angiography, 2.5−20 months (mean 9.1) after dilation, showed an increase in the diameter of the outflow tract from 7.1±2.1 mm before to 9.5±2.0 mm after dilation (p<0.05). Diameters of the pulmonary arteries increased from 6.3±2.2 mm on the right and 6.8±1.9 mm on the left before to 9.2±2.2 mm and 9.3±2.5 mm respectively after dilation (p<0.05 for both branches). Surgical correction has been achieved in 22 patients, 21 of these 2−22 months after balloon dilation and the other patient 20 months after construction of a shunt. At operation, the leaflets of the pulmonary valve were noted to be torn in five patients. In 12 patients, there was extensive scarring of the infundibulum. Balloon dilation of the right ventricular outflow tract achieves adequate palliation and may result in an increase in the diameter of the pulmonary valve and branches of the pulmonary trunk. It should be considered as an alternative to shunt procedures in children with tetralogy of Fallot.
Total correction of tetralogy of Fallot without “routine”preoperative cardiac catheterization—management of 99 patients
Authors: Sarioglu Ayse , Batmaz Gülhis , Bilal Mehmet Salih , Saltik Irfan Levent , Saylam Gül , Sarioglu Tayyar , Aytac Aydin , Ertugrul Ali
Published: August 2008

Between January 1989 and March 1993, total correction was performed in 99 patients with tetralogy of Fallot without submitting them to prior cardiac catheterization. The age of the patients ranged from 1.33 to 18 years (mean 5.33±3.77). After complete echocardiographic examination, the diameters of the right and left pulmonary arteries at the prebranching point and the descending thoracic aorta at the diaphragm were measured by cross-sectional echocardiography and the McGoon ratio was calculated. Total correction was performed in all patients with a McGoon ratio greater than 1.7. In none of the patients were the sizes of the pulmonary artery measured by echocardiography smaller than the measurements obtained during surgery. Transannular patching was performed in 76 patients. A conduit from the right ventricle to the pulmonary arteries was constructed in two patients with coronary arterial anomalies. Postrepair right ventricular to left ventricular systolic pressure ratios were between 0.25 and 0.85 (mean 0.54±0.13). There were two hospital deaths, neither being related to the diagnostic method used nor the criteria for surgery. We conclude that the diagnosis of tetralogy of Fallot together with measurements of pulmonary arteries and descending thoracic aorta can safely and reliably be achieved echocardiographically. The McGoon ratio can be adapted to echocardiography and total correction can be performed successfully based on echocardiographic examination.
Deposition of collagen in the alveolar wall of lungs from patients with tetralogy of Fallot and pulmonary atresia with major aortopulmonary collateral arteries—an ultrastructural study
Authors: Matsuda Hikaru , Kuratani Tohru , Shimazaki Yasuhisa , Kadoba Keishi , Kobayashi Jyunjiro , Ohtake Shigeaki , Nakayama Masahiro , Nakada Takeshi
Published: August 2008

Abstract Lung biopsies were taken at surgery from five patients (age 2–13, average 7.6 years) with tetralogy of Fallot and pulmonary atresia with major aortopulmonary collateral arteries. The biopsies were then processed for ultrastructural study, comparing paired samples taken, on the one hand, from a segment connected to central pulmonary arteries and, on the other hand, from a segment supplied directly by collateral arteries. Specimens from patients with isolated ventricular septal defect, and from those without cardiac disease, were used as controls. In the patients with tetralogy of Fallot and pulmonary atresia, all biopsies taken from segments supplied by the major collateral arteries showed marked deposition of collagen in the alveolar wall, with an increase in the thickness of the basement membrane (3.6±1.2 µm, mean±SD) greater than seen in those taken from segments connected to central pulmonary arteries (0.9±0.6, p<0.05). The proportional fibrosis of the alveolar interstitial space was also significantly greater in the biopsies from the segments supplied by collateral arteries (30±9%) compared to those from segments fed by central pulmonary arteries (15±8%, p<0.05). No significant differences were found in these indices between the biopsies from segments connected to the central pulmonary arteries in patients with tetralogy of Fallot and pulmonary atresia and those from patients with isolated ventricular septal defect or normal controls. There was no apparent relation to the pulmonary arterial pressure in these findings. The results suggest that the pulmonary segments fed directly by major aortopulmonary collateral arteries in patients with tetralogy of Fallot and pulmonary atresia seem likely to be afflicted by alveolar wall fibrosis, although the etiology and clinical implications of this finding remain unclear.
Dimensions of the pulmonary arteries in rat fetuses with congenital heart disease
Authors: Momma Kazuo , Ando Masahiko , Yoshigi Masaaki
Published: August 2008

So as to understand better the pathogenesis of enlargement or hypoplasia of pulmonary arteries in congenital heart disease, we studied the dimensions of the pulmonary arteries in 74 fetuses with congenital heart disease induced by administration of bis-diamine to pregnant rats. The congenital malformations induced included 12 with large ventricular septal defect, 17 with tetralogy of Fallot, 15 with tetralogy together with severe valvar pulmonary stenosis and absence of the arterial duct, 17 with tetralogy with absent pulmonary valve syndrome and absence of the arterial duct, and 13 with common arterial trunk with a confluent segment supplying the pulmonary arteries. For comparison, 16 fetuses of the same gestational age with normal hearts were studied. After rapid whole-body freezing on the 21st day of gestation, the fetuses were studied by means of serial cross-sectional photographs of the thorax. The diameter of the right pulmonary artery of the fetus was of comparable dimensions in the normal hearts (480±10 µm) (mean±SEM), those with ventricular septal defects (470±10 µm), common arterial trunk (520±20 µm), and tetralogy of Fallot (500±10 µm). These findings suggest that the commonly observed enlargement of the right pulmonary artery in patients with ventricular septal defect and common arterial trunk, and hypoplasia of the right pulmonary artery in tetralogy of Fallot, occur postnatally in response to abnormal postnatal pulmonary blood flow.
Aortic valve replacement in tetralogy of Fallot and pulmonary atresia with major aortoputmonary collateral arteries
Authors: Kito Hiroyuki , Yagihara Toshikatsu , Kawashima Yasunaru
Published: August 2008

A 29-year-old woman who had tetralogy of Fallot and pulmonary atresia with major aortopulmonary collateral arteries developed aortic regurgitation due to infectious endocarditis. Isolated aortic valve replacement was undertaken because of heart failure unresponsive to medical therapy. Excessive flow through the aortopulmonary collateral arteries during cardiopulmonary bypass was controlled by means ofintraaortic balloons which were inflated to occlude the origin of the collateral arteries. Arterial return from the pump was delivered through separate cannulation of the ascending aorta and the right femoral artery. We believe that this method should be considered in those circumstances when excessive flow through systemic-to-pulmonary arteries is expected during cardiopulmonary bypass.
Transient complete heart block following balloon pulmonary valvoplasty in a case of congenitally corrected transposition
Authors: Romero Leopoldo , Sigwart Ulrich , Somerville Jane
Published: August 2008

Balloon pulmonary valvoplasty was performed in a patient with congenitally corrected transposition, aged 28 years, who had already undergone surgical closure of a ventricular septal defect and implantation of a conduit between the pulmonary arteries and the right-sided morphologically left ventricle. The patient developed stenosis of the conduit 22 years after the procedure as confirmed by cardiac catheterization and angiocardiography, which also showed stenosis of the native pulmonary valve. A balloon valvoplasty of the native valve was carried out with improvement of the gradient from the ventricle to the pulmonary arteries. The patient developed complete heart block immediately after the procedure, which reverted to first degree block in one week and to the previous cardiac rhythm within 21 days.
Interrupted fourth aortic arch with persistent fifth aortic arch and aortic coarctation—treatment with balloon angioplasty combined with surgery
Authors: Jurí Raul , Alday Luis Eduardo , De Rossi Roberto
Published: August 2008

A symptomatic neonate was referred for aortic coarctation and, at cineangiography, was shown to have an interruption of the aortic arch at the isthmus together with coarctation related to a persistent fifth aortic arch. Balloon dilation of the coarctation was performed, but the coarctation was still evident after six months. A graft was then interposed between the left subclavian artery and the descending aorta. Balloon dilation was repeated five years later leaving a residual gradient of 11 mm Hg.
Fixed subaortic stenosis and redundant mitral valve forming subaortic chamber during ventricular systole
Authors: Lorber Abraham , Itzkovitch D. , Hallon F.
Published: August 2008

A 43-year-old female with complaints of exertional dyspnea was found by Doppler echocardiography to have fixed subaortic stenosis and a redundant mitral valve which, during systole, formed a chamber in the left ventricular outflow tract. This resulted in a subaortic gradient of 40 mm Hg. There was also moderate aortic regurgitation.
News and comments
Authors: Anderson Robert H. , Henry G. William , Crupi Giancarlo
Published: August 2008

HAPPY NEW YEAR! IT BECOMES INCREASINGLY EVI-dent that it is a truism that years go past quicker as we become older. Ironically, as time goes by faster, we must devote more of our attention to the future. For readers of Cardiology in the Young, as we enter our fifth year of publication, the future is indeed bright. As of this issue you join readers on six continents and scores of countries. As editors, we are most grateful for your continued support. You will notice changes that we are incorporating into this volume, beginning with a complete redesign of our cover and minor changes in the size of the journal. Further substantive editorial changes will be incorporated throughout the year, and we will keep you fully informed as we go. Exciting changes will occur as we augment participation from colleagues in Japan, China and throughout Asia. Please share with us your ideas, as we are amenable to any change or addition that will further improve the quality of Cardiology in the Young.
Shunts and security—the polytetrafluoroethylene leak
Authors: McKay Roxane
Published: August 2008

Plasma leakage in polytetrafluoroethylene grafts arises through complex interactions between the prosthetic physical/chemical microstructure and patient environment. While many of the precipitating factors can be avoided by careful graft-handling and meticulous surgical technique, this unpredictable complication still contributes significant morbidity to shunt procedures. Protein sealing of the graft offers a possible solution to the problem.
Long-term follow-up of treated critical aortic stenosis
Authors: Kitchiner Denise , Sreeram Narayanswami , Malaiya Nilima , Jackson Mark , Walsh Kevin , Peart Ian , Arnold Robert
Published: August 2008

To determine the long-term results in patients with critical aortic stenosis who survive initial intervention, and to identify factors which predict prognosis, we studied patients who underwent intervention between 1979 and 1992 for critical aortic stenosis treated within the first three months of life. Patients with a hypoplastic left ventricle or mitral stenosis who were not considered for a biventricular repair were excluded. Follow-up examination included cross-sectional and Doppler echocardiography. All initial and subsequent patient data were reviewed. Of the 64 patients with critical aortic stenosis, 41 (64%) survived more than one month after initial intervention (surgical valvotomy in 39, balloon valvoplasty in two). These survivors constitute the study group. Mild or moderate residual aortic stenosis or regurgitation without further intervention was found in 28 patients at a median duration of 3.1 years (range 0.2–15.0 years). A poor result with re-intervention (n=6) or death (n=7) occurred in 13 patients. The diameter of the aortic valve at presentation was smaller (p<0.02) in patients with a poor result (median 5.5; range 5–15 mm), than in those with a satisfactory result (median 8.0; range 5–10 mm). Significant residual aortic stenosis was present from the time of initial intervention in nine of the 13 patients (69%) with a poor result. No difference was found in the incidence of a duct-dependent systemic circulation, associated cardiac lesions, mechanical ventilation, acidosis or the use of inotropes preoperatively between patients with a satisfactory or a poor late outcome. Of patients with critical aortic stenosis, 64% survived for more than a month after initial intervention. A small aortic valvar diameter at presentation ( 6 mm) and residual stenosis after initial intervention were important determinants of long-term prognosis.
One-stage repair for intracardiac malformations associated with interrupted aortic arch or aortic coarctation in the first year of life
Authors: Matsuki Osamu , Yagihara Toshikatsu , Yamamoto Fumio , Nishigaki Kyoichi , Uemura Hideki , Kagisaki Koji , Takahashi Osahiro , Kamiya Tetsuro , Kawashima Yasunaru
Published: August 2008

A one-stage repair was performed for correction of the intracardiac malformations associated with coarctation of aorta in five pateints or interrupted aortic arch in eight patients. The ages ranged from four to 294 (median 35) days. The anomalies within the heart were a ventricular septal defect with or without subaortic stenosis (n=7), an aortopulmonary window (n=2), common arterial trunk (n=2), aortic valvar stenosis (n=1) and the TaussigBing anomaly (n=1). Surgery was performed through a median sternotomy employing cardiopulmonary bypass with moderate to deep hypothermia. In terms of the aortic reconstruction, an extended direct anastomosis was performed in 10 patients, while a vascular graft was interposed in three. So long as the aortic cannula did not interfere with the proximal anastomotic site on the aorta, circulatory arrest was avoided. As for surgery within the heart, the ventricular septal defects were closed via the right atrium with myotomy and myectomy if a morphological substrate for subaortic stenosis was confirmed (n=4). There were two hospital deaths (15.4%) due to low cardiac output. In patients who underwent myotomy and myectomy for subaortic stenosis, the postoperative pressure gradient across the aortic valve was negligible. We conclude that surgical results of one-stage repair for the intracardiac malformations associated with interrupted aortic arch or aortic coarctation are reasonable. We suggest that the early relief of obstruction within the left ventricular outflow tract may have played some role in the favorable outcome.