bluuebunny/crossref_metadata_2025 huggingface dataset:
Pulmonary atresia with intact ventricular septum: management of the coronary arterial anomalies
Authors: Dyamenahalli Umesh , Hanna Brian D. , Sharratt Geoffrey P.
Published: August 2008

Over a period of 12 years, we encountered 30 cases of pulmonary atresia with intact ventricular septum. The overall mortality was 53% (16 patients). Coronary arterial abnormalities were detected in 16 patients and, in 10 of these, the coronary arterial circulation was partially or totally dependent on the right ventricle. The mortality in this group was 80%. The strategies for management are reviewed in 6 cases to try to define the best approach to diagnosis, and to assess the benefits of the various modesof treatment used for specific coronary arterial anomalies. Two cases (2 and 3) underwent occlusion of a fistula from the right ventricle to a coronary artery using embolization coils. This modality is considered to be appropriate and useful when the coronary circulation supplied by the fistula is not dependent on flow from the right ventricle. The dependency of the coronary arterial circulation on the right ventricle was extensive in two cases (Case 4 and 6), and complete in one because of atresia of the orifices of the coronary arteries (Case 5). These three patients died. Cardiac transplantation is suggested as the most appropriate treatment in this group. In two cases (1 and 6), there was a supernumerary vessel connecting the right ventricle to the pulmonary trunk. These connections do not appear to be of therapeutic concern, as they do not seem to contribute to myocardial blood supply. The selection of the most appropriate protocol for management of patients with coronary arterial anomalies depends upon a thorough and aggressive approach to investigation.
Successful dilation of superior cavel venous obstruction following repair of totally anomalous pulmonary venous connection
Authors: Shrivastava S. , Radhakrishnan S. , Chandra Y. Vijaya
Published: August 2008

We describe post azygos obstruction of the superior caval vein following repair of a mixed variety of totally anomalous pulmonary venous connection. Balloon dilation was immediately successful, and has provided persistent relief at a follow-up of 20 months.
Absent pulmonary valve in tricuspid atresia with left ventricular outflow tract obstruction and patent arterial duct
Authors: Lemke Robert P. , Giddins Neils G. , Odim Jonah N.K.
Published: August 2008

We describe a neonate with tricuspid atresia, absent pulmonary valve, right ventricular hypoplasia, small venrricular septal defect, left ventricular ourflow tract obstruction and a patent arterial duct. These finding were diagnosed by echocardiography and confirmed by cardiac catherterization and postmortem examination. This is a unique report of a functionally single ventricle variant of absent pulmonary valve syndrome and biventricular outlet obstuction.
Sequential segmental analysis - description and categorization for the millennium
Authors: Anderson Robert H. , Yen Ho Siew
Published: August 2008

The concept of sequential analysis of congenitally malformed hearts is now widely accepted, although some aspects remain controversial. In this review, we analyse the evolution of the scgmental approach, showing how our own concepts of analysis have chaanged little since our initial description, heart dicrates the presence of the atrial, ventriclar, and arterial segments, Analysis depends upon recognizing the morphologic differences of the components within each segment, and then how these parts are joined together across the atrioventicular and ventriculo-arterial Junctions. The distinction of chambers and arterial trunks follows the principle of the morphologic method, namely that scructures be identified on the basis of their own intrinsic morphology, and not according to other features which themselves might be variable. Differentiation of junctional arrangements as opposed to segmental stucture resolves ongoing controversics such as the nature of “univentricular hearts”.Analysis in the fashion proposed shows that there is nothing intrinsically complicated with malformed hearts.
Morphology of hearts undergoing Fontan repair
Authors: Murari Vivek , Sharma Rajesh , Airan Balram , Bhan Anil , Choudhary Shiv K. , Saxena Anita , Kothari Shyam S. , Reddy Subhash C. B. , Venugopal Panangipalli
Published: August 2008

Background

Notwithstanding the controversies evoked by the term “single ventricle”, most patients with this condition would undergo the Fontan procedure. In addition, there is a large group of patients in whom a biventricular repair would be abandoned in favour of a univentricular one because of the presence of unfavourable morphologic features. There is a need for a uniformly acceptable system of nomenclature that would permit precise description and classification of hearts with complex malformations to facilitate reporting and help in understanding the reasons for choosing a univentricular repair.

Methods

Echocardiographic, angiographic and operative records of 240 patients undergoing the Fontan procedure were analysed.

Results

Out of the 104 patients with univentricular atrioventricular connections, 2 ventricles were discernible in all but 3 patients. A Fontan repair was performed in 136 patients with biventricular atrioventricular connections because of the presence of a hypoplastic ventricle in 52 patients and a non-committed ventricular septal defect in the remaining 84.

Conclusions

The Fontan operation is probably the only definitive treatment option for patients with univentricular atrioventricular connections. The decision to perform a univentricular repair in preference to a biventricular one in hearts with biventricular atrioventricular connections is based on the presence of a hypoplastic ventricle or a non-reroutable ventricular septal defect. This decision is subjective. In hearts with discordant atrioventricular connections and pulmonary stenosis, we prefer the Fontan operation to the classical repair.
Anomalies of the aortic arch and arterial duct induced by small doses of bis-diamine in fetal rats
Authors: Momma Kazuo , Ando Masahiko
Published: August 2008

Deletion of chromosome 22 and DiGeorge syndrome are associated with inappropriate development of the neural crest and abnormalities of the ventricular outflow tracts. Bis-diamine induces comparable cardiac anomalies by inhibiting the normal development of the neural crest. In order to clarify the effect of damage on the development of the neural crest and its relation to the cardiac anomalies, the effects of giving a small dose of bis-diamine were compared with the results of a large dose of bis-diamine. A small dose of bis-diamine (20 mg), therefore, was administered to 40 pregnant rats on the 10th day of pregnancy, and 330 fetal hearts were studied on the 21st day with the rapid whole-body freezing method. Intracardiac anomalies were rare, being found in only 55 fetuses. The anomalies discovered were: aberrant origin of the subclavian artery in 88 fetuses, right aortic arch in 26 fetuses, interruption of the aortic arch in 23 fetuses, and high location of the aortic arch in 8 fetuses. Anomalies of the arterial duct were also common; with a vascular ring formed by the aortic arch and contra-lateral duct being seen in 21 fetuses, isolation of the right subclavian artery associated with either bilateral ducts (9 fetuses) or a right duct and absent left duct (9 fetuses), absent duct with tetralogy of Fallot in 7 fetuses, and with common arterial trunk in one fetus. In conclusion, a small dose of bis-diamine induced extra cardiac anomalies of the aortic arch and arterial duct, indicating a greater susceptibility of the aortic arch and the duct to inappropriate formation of the neural crest cells in the early developing embryo. Clinically, these anomalies of the aortic arch and the duct should be searched for in all patients suspected of having deletion of their 22nd chromosome.
The spectrum of pulmonary input impedance in children with complete transposition after the arterial switch operation
Authors: Murakami Tomoaki , Nakanishi Toshio , Nakazawa Makoto , Momma Kazuo , Imai Yasuharu
Published: August 2008

We evaluated the physical characteristics of the pulmonary trunk in 32 patients with complete transposition after the arterial switch operation, calculating the pulmonary input impedance. In 6 of the patients, the characteristic impedance was equal to, or less than, the value in patients with ventricular septal defect. In those patients with an intact ventricular septum, or those with a ventricular septal defect who had undergone the arterial switch operation or banding of the pulmonary trunk before 3 months age, the frequency of the first modulus minimum also agreed with the values obtained in patients with ventricular septal defect. The value was high, however, in patients with complete transposition with ventricular septal defect who had undergone an arterial switch operation or banding of the pulmonary trunk after 4 months of age. Our data suggest decreased distensibility of the proximal pulmonary trunk after the arterial switch operation, and mild changes in the pulmonary vasculature in patients with complete transposition with ventricular septal defect who had undergone either an arterial switch operation or banding of the pulmonary trunk after 4 months of age. Long-term follow-up is needed clarify whether or not these features affect right ventricular function by means of an increase in afterload.
Balloon dilation for postoperative pulmonary stenosis following surgical creation of an intrapulmonary coronary arterial tunnel
Authors: Mori Yoshiki , Nakanishi Toshio , Nakazawa Makoto , Momma Kazuo , Imai Yasuharu
Published: August 2008

Balloon dilation was performed in four patients with postoperative pulmonary stenosis who had undergone surgical creation of a coronary arterial tunnel in the pulmonary trunk. Two patients had complete transposition in whom the arterial switch operation had been performed using the modified Aubert method. The other two patients had anomalous origin of the left coronary artery from the pulmonary trunk treated with the Takeuchi procedure. Balloon dilation was performed at 11 locations. The pressure gradient decreased from 48 ± 22 to 24 ± 14mmHg (p<0.01), and the diameter of the narrowest segment increased from 5.3 ± 2.5 to 7.5 ± 2.8mm (p<0.01), respectively. Of the 11 procedures, 8 (73%) were judged successful with use of the criterion of success as a greater than 50% decrease in pressure gradient, and/or a greater than 50% increase in diameter. The inflated balloon must have compressed the coronary arterial tunnel in the pulmonary trunk, but there was no apparent myocardial damage in any patient, although transient and mild ST-T changes appeared on electrocardiographic monitoring during the procedure in 2 patients. Rupture of the wall of the pulmonary trunk occurred in two patients, one of whom required elective surgical intervention. These data suggest that balloon dilation should be performed with caution for management of postoperative pulmonary arterial stenosis in patients with a surgically created intrapulmonary coronary arterial tunnel, since tearing the wall of the pulmonary trunk may occur.
Factors that predict epinephrine induced constriction of the persistent arterial duct
Authors: Tomita Hideshi , Fuse Shigeto , Chiba Shunzo
Published: August 2008

We studied factors that predict in children catecholamine induced vasoconstriction of the persistent arterial duct. Epinephrine at 2.0–10.0 (4.9 ± 1.3, mean ± standard deviation) μg/kg was injected intravenously into 30 patients with persistent arterial duct whose age ranged from 1 to 91 (27 ± 26) months. In 11 patients aged 10–66 (34 ± 23) months (responders), a continuous murmur had almost completely disappeared on auscultation, and both the diameter and the shunt flow area of the arterial duct had become smaller as shown by Doppler echocardiography. On the other hand, there was no such change in 19 patients aged 1–91 (23 ± 27) months (non-responders). Although there was no significant difference in the age, the body weight, the minimal diameter of the arterial duct, or the doses of epinephrine between responders and non-responders, the gestational age at birth was slightly less (p=0.09) and the birth weight was significantly smaller in responders than in non-responders (p<0.05). The persistent arterial duct of those who had a history of low birth weight always responded to epinephrine. In infants younger than 6 months, the persistent arterial duct was never constricted by epinephrine regardless of the birth weight and gestational age. In low birth weight infants, the vascular smooth muscle of the persistent arterial duct can usually constrict beyond infancy. There may be some age dependent difference in responsiveness to epinephrine until 6 months of age.
Chylothorax after surgery for congenital heart disease in children
Authors: Pedra Simone Rolim Fernandes Fontes , Pedra Carlos Augusto Cardoso , Fontes Valmir Fernandes , Santana Maria Virgínia Tavares , Paulista Paulo Paredes
Published: August 2008

We reviewed retrospectively the clinical and surgical data from 9 children with postoperative chylothorax secondary to cardiac surgery for congenital heart disease seen over a 3 year period. Mean age was 26 months and 6 patients were female. The procedures performed were 2 Blalock-Taussig shunts, 6 Bidirectional Glenn anastomoses and 1 modified Fontan procedure. Diagnosis of chylothorax was made by classical means. The overall incidence of chylothorax was 0.8%, with the relative incidences being 2.5% for Blalock-Taussig, 2.7% for Fontan, and 11.7% for the Glenn procedures. All patients were initially treated conservatively with chest tube drainage and hypercaloric diet suplemented with medium chain triglycerides. Parenteral nutrition was needed in 2 patients, one because of sepsis and the other because of progressive malnourishment. High output fistulas and long periods of drainage were noted after cavo-pulmonary procedures. Up to 12% weight loss was observed in 6 patients, one patient was successfully treated of pneumonia and another died ofPseudomonas aeruginosasepsis. Two patients required chemical pleurodesis because of unabated drainage, with the others being successfully treated conservatively. Chylothorax is a common complication after bidirectional Glenn procedures, and is associated with considerable morbidity and mortality. Earlier operative intervention may be required in these patients. Pleurodesis is a simple, safe, and effective procedure to stop chylous flow.
Beneficial effect of an oral analog of prostacyclin on pulmonary hypertension secondary to congenital heart disease
Authors: Ichida Fukiko , Uese Kei-ichiro , Tsubata Shin-ichi , Hashimoto Ikuo , Hamamichi Yuji , Fukahara Kazuaki , Murakami Arata , Miyawaki Toshio
Published: August 2008

To determine whether a newly synthesized oral analog of prostacyclin, called beraprost sodium, could cause pulmonary vasodilation, we studied its hemodynamic effect on pulmonary hypertension of children, comparing it to other vasodilatory agents, such as nitric oxide and tolazoline. We studied 20 children (mean age 24 months) having pulmonary hypertension secondary to congenital heart disease. A single oral dose of beraprost sodium resulted in an appreciable reduction of pulmonary vascular resistance (mean 34%), which was comparable to that induced by inhalation of nitric oxide and intravenous delivery of tolazoline (mean 41% and 31%, respectively). The results suggest that beraprost sodium may serve as a novel and safe vasodilator for the screening of pulmonary vasoreactivity, as well as the treatment of pulmonary hypertension in children.
Formation of thrombus and thromboembolism after the bidirectional Glenn anastomosis, total cavopulmonary connection and the Fontan operation
Authors: Hanséus Katarina , Björkhem Gudrun , Jögi Peeter , Sonesson Sven-Erik
Published: August 2008

Although patients undergoing surgery with the Fontan procedure or its modifications are increasingly recognised to be at risk for thromboembolism, further knowledge is needed to minimise this complication and its sequels. To address this issue, we reviewed 100 patients operated with the Fontan procedure, the bidirectional Glenn anastomosis and/or the total cavopulmonary connection to describe our incidence and clinical characteristics of postoperative formation of thrombus. Symptomatic thrombosis or cerebrovascular accidents were found in 5 patients. Asymptomatic thrombus were found in another 5 patients. Three patients had venous thrombi. In 6 patients the thrombosis was found on the arterial side. In all these cases, the thrombus was located in the stump of the divided pulmonary trunk. In 3 of these patients, sudden onset of hemiparesis preceded the diagnosis of the thrombus while the remaining 3 patients were asymptomatic. In 3 cases, the formation of thrombus in the stump of the divided pulmonary trunk occurred after a bidirectional Glenn anastomosis. One patient developed severe neurological symptoms 2 months after a total cavopulmonary connection. No thrombus was found, but the patient had a small right-to-left shunt and embolization could not be excluded. The incidence of thrombosis after Fontan-type surgery in this study is 10%. Although not all episodes of thrombosis are symptomatic, there is a significant risk of severe sequels due to embolization to the pulmonary or cerebral circulations. The stump of the divided pulmonary trunk is one of the main sites for intracardiac formation of thrombus, even after a bidirectional Glenn anastomosis.
Surgical repair of interrupted aortic arch with ventricular septal defect
Authors: Allan Lindsey D , Apfel Howard D , Levenbrown Yosef , Quaegebeur Jan M
Published: August 2008

Background

Interrupted aortic arch is often associated with subaortic narrowing and hypoplasia of the aortic orifice. The best surgical strategy for the management of these additional lesions is a matter of current debate.

Methods and Results

Between 1986 and 1996, 19 patients underwent repair of interrupted aortic arch with closure of ventricular septal defect in a single stage, with no attempt at subaortic resection, irrespective of the dimensions of the left ventricular outflow tract. There was no perioperative hospital mortality, and all patients were alive at 1 year. Follow-up ranges from 0.75 −10 years, with a mean 4.2 ± 3.0 years. Seven patients (37%) have required reintervention for relief of subaortic stenosis, 2 of whom have died.

Conclusions

Our results suggest that primary one-stage biventricular repair can be accomplished with low perioperative mortality without addressing the subaortic region. Further long-term follow-up will determine whether this is accomplished at the expense of later morbidity and mortality.
Cavo-pulmonary anastomosis excluding the portal venous return: an alternative for patients with protein-losing enteropathy secondary to the Fontan procedure
Authors: Barbero-Marcial Miguel , Riso Arlindo , Atik Edmar , Tanamati Carla , Reis Humberto , Sobrinho Veloso , Jatene Adib
Published: August 2008

Severe protein losing enteropathy occurred in 3 of our 128 patients undergoing the Fontan procedure in the period of 1982 to 1994. The complication occurred 4, 4.5 and 5 years after the procedure, respectively. One patient had double inlet left ventricle while the other two had tricuspid atresia. All had initially undergone an atrio-pulmonary connection, and the duration of the enteropathy was 12, 13,5 and 15 months. All patients were in functional class IV of the New York Heart Association. The presence of protein losing enteropathy was confirmed by determining levels of fecal alfa-1 antitrypsin, enteric clearance of 51 Cromo labeled albumin, and endoscopy with histopathological study of the small bowel.

They underwent further surgery to convert the atrio-pulmonary to a cavo-pulmonary anastomosis which excluded all the hepatic veins. A bovine pericardial baffle was sutured in the inferior caval vein so as to leave the hepatic venous flow draining to the right atrium and thence through an atrial septal defect to the left atrium and systemic circulation. The inferior caval venous flow was directed through an intra-atrial tunnel to the right pulmonary artery, and the superior caval vein was anastomosed to the superior aspect of the right pulmonary artery, thus completing the total cavo-pulmonary anastomosis with exclusion of the portal venous flow.

Postoperatively, there was total regression of the clinical and laboratory findings. All patients are now in functional class I or II of the New York Heart Association. They have mild to moderate cyanosis up to three and a half years from the reoperation. We recommend the technique as an alternative for patients with severe protein losing enteropathy occurring after the Fontan procedure.
Contrast echocardiography for diagnosis of pulmonary arteriovenous fistulas late after construction of a Glenn anastomosis
Authors: Sagin-Saylam Gül , Somerville Jane
Published: August 2008

To demonstrate the use of transthoracic contrast echocardiography in the detection of pulmonary arteriovenous fistulas in patients with a previously constructed anastomosis between the superior caval vein and the right pulmonary artery (Glenn shunt), and to examine their prevalence in this special population, we evaluated prospectively all patients followed up in the Grown-Up Congenital Heart Unit subsequent to construction of a classical or bi-directional Glenn shunt. We studied 12 patients, aged from 21 to 38 (mean 28 ± 4.8) years who had had a previous cavopulmonary shunt in place for a period of 4 to 33 years (mean 24±9 years). All were examined with cross-sectional contrast echocardiography, 11 patients had cardiac catheterisation and angiography, and 6 patients had magnetic resonance imaging. Systemic arterial oxygen saturations at rest, and during exercise using the modified Bruce protocol, were measured in all patients. Contrast echocardiography showed evidence of pulmonary arteriovenous fistulas in 7 of the 12 patients, with appearance of echo contrast in the left atrium 1–8 seconds after peripheral venous injection in the arm. Simultaneous appearance of microbubbles in the right atrium revealed a residual communication between the superior caval vein and the right atrium in 2 patients, and presence of collaterals between the superior and inferior caval veins in one. Cardiac catheterisation and angiography showed obvious fistulas in 4 patients, and revealed suggestive findings in 2. In patients deemed to have pulmonary arteriovenous fistulas on contrast echocardiography, arterial oxygen saturations at rest (51–94%, mean 75±15.3%) and on exercise (23–91%, mean 53±24.2%) were significantly lower compared to patients judged to be without fistulas (p<0.005). Pulmonary hypertension in the contralateral lung was more common in patients with fistulas (mean left pulmonary arterial pressure 22–110 mm Hg, p=0.014). In patients with cavopulmonary anastomoses, pulmonary arteriovenous fistulas occur frequently in the long term (10–33, mean 25.7±8 years), and are associated with worsening systemic arterial desaturation. Contrast echocardiography should be included in the regular evaluation of these patients as a simple and sensitive technique for the detection of pulmonary arteriovenous fistulas, particularly with the devel opment of increasing cyanosis.
Criss-cross heart with supero-inferior ventricles: successful surgical correction of associated defects
Authors: Kaushal Sunil K. , Shrivastava Savitri , Iyer Krishna S.
Published: August 2008

We present an unusual case of criss-cross heart with supero-inferior ventricles, double outlet of the right ventricle, restrictive ventricular septal defect, severe infundibular stenosis and “absent” pulmonary valve who underwent successful surgical repair. Complete preoperative diagnosis was established with cross-sectional echocardiography combined with colour Doppler and angiocardiography, which facilitated successful surgical correction.
Noninvasive diagnosis of coronary arterial fistulas using multiplanar cine magnetic resonance imaging
Authors: Tsubata Shin-ichi , Ichida Fukiko , Miyawaki Toshio
Published: August 2008

In three children with coronary arterial fistulas, two extending from the right coronary artery to the right atrium and one from the left circumflex coronary artery to the right ventricle, multiplanar cine magnetic resonance imaging was able accurately to demonstrate dilation of the involved coronary artery, the tortuous nature of the dilated fistulas, blood flow within the fistulas, and luminal narrowing. The technique provides a new adjunct for management of patients, especially young children, with coronary arterial fistulas.
Repair of divided left atrium associated with unroofed coronary sinus
Authors: van Son Jacques A.M. , Walther Thomas , Mohr Friedrich W.
Published: August 2008

In a young infant with divided left atrium, unroofed coronary sinus syndrome, and persistent left superior caval vein, the pulmonary venous blood was successfully routed, after atrial septectomy, to the mitral valve by constructing a baffle using the partially detached diaphragm which had initially separated the pulmonary venous and vestibular components of the left atrium. The baffle was augmented with a patch of untreated autologous pericardium.
Right ventricular biopsy findings of hypertrophic cardiomyopathy in a patient with Noonan syndrome
Authors: Skinker Donna M. , Cottrill Carol M. , O'Connor William N.
Published: August 2008

We report right ventricular ultrastructure in a 20-year-old patient with Noonan's Syndrome. Initial left ventricular and septal myocardial hypertrophy, observed at 2 months without obstruction, progressed to biventricular involvement over 18 years. Histopathologic evidence of cardiomyocytic disarray, with ultrastructural evidence of myofibrillar disarray, confirmed typical biopsy findings of hypertrophic cardiomyopathy on the right ventricular side of the septum.
Anomalous origin of the right coronary artery from the pulmonary trunk
Authors: Pinto Fátima Ferreira , Kaku Sashicanta , Ramos José Sousa
Published: August 2008

We report a case of anomalous origin of the coronary artery from the pulmonary trunk. The lesion was discovered in an eleven-month-old asymptomatic boy because of a continuous murmur.
An unusual anomalous course of a coronary artery from the pulmonary trunk, coexisting with congenital mitral stenosis and aortic coarctation
Authors: Ho Siew Yen , Gatzoulis Michael A. , Sheppard Mary
Published: August 2008

Anomalous origin of a coronary artery is occasionally associated with other congenital heart malformations. Presence of the anomalous artery can then complicate surgical repair. We report an unusually complex arrangement in a patient who died 16 hours following complete surgical repair despite appropriate management.
A complex variant of tricuspid atresia: survival to 60 years without surgery
Authors: Gerlis L M , Mayet J , Somerville J
Published: August 2008

We report a case of tricuspid atresia in which the symptoms underwent spontaneous improvement during childhood, and did not return until the age of 56 after which the patient went into progressive congestive failure and atrial fibrillation. He died at the age of 60, without ever having surgical intervention. Autopsy confirmed the presence of tricuspid atresia due to absence of the right atrioventricular connection, and also revealed partial left juxtaposition of the atrial appendages, an atrial septal defect in the oval fossa, rudimentary right ventricle, double outlet left ventricle with anterior aorta (anatomically corrected malposition), subvalvar and valvar pulmonary stenosis, dilation of the pulmonary arteries, patent arterial duct and major systemic to pulmonary collateral arteries.

It was considered that the development of the systemic to pulmonary arterial anastomoses probably accounted for the initial remission of symptoms, whilst the obstruction of the pulmonary outflow tract prevented the development of any significant degree of pulmonary hypertension in the presence of double outlet left ventricle.
Implantation of endovascular stents for hypoplasia of the transverse aortic arch
Authors: Pihkala Jaana , Pedra Carlos A. C. , Nykanen David , Benson Lee N.
Published: August 2008

Hypoplasia of the transverse aortic arch is commonly associated with aortic coarctation. Persistent or recurrent obstruction can occur at this level after successful repair of the native coarcted segment. The purpose of this report is to present a new technique to treat such lesions, namely with implantation of a balloon-expandable stent. This approach was used successfully in 4 children with such hypoplasia occurring after repair of coarctation. Implantation led to both anatomical and physiological relief of obstruction in all. The patients tolerated the procedure, and there were no major adverse events.
Cyanotic nephropathy and use of non-ionic contrast agents during cardiac catherization in patients with cyanotic congenital heart disease
Authors: Dittrich Sven , Kurschat Katrin , Dähnert Ingo , Vogel Michael , Müller Christian , Lange Peter E.
Published: August 2008

Background

Chronic cyanosis with its associated rheologic changes is a known risk factor for glomerular nephropathy. Therefore, contrast-induced nephrotoxicity should be an important consideration for angiographers comparable to diabetics. On the other hand, progressions in diagnostic and interventional techniques have led to expanded indications and a more widespread use of x-ray contrast agents. The aim of this study was to investigate the risk of contrast-induced nephropathy in the small group of patients with cyanotic heart disease prior to surgical repair.

Methods

We investigated 23 cyanotic patients with an oxygen saturation of 82 (50–92)%, age 25 (5–63) years, and 13 control subjects with atrial septal defect, age 37 (20–66) years. Blood viscosity was measured before and after cardiac catherization. Renal damage was evaluatated by selective analysis of urinary proteins and enzymes.

Results

Before cardiac catheterization, 48% of the cyanotic patients had a moderate glomerulopathy. Cardiac catherization was performed with 3.0 (1.2 – 6.8) mls/kg non ionic contrast medium. Only one of the 23 patients (4.3%) with normal urinary analysis before cardiac catheterization showed renal damage, which involved tubular and glomerular function. Elevated blood viscosity in cyanotic patients was slightly reduced by the contrast. None of the acyanotic controls had contrast-induced nephropathy.

Conclusions

The use of non-ionic contrast medium does not worsen cyanotic glomerulopathy. This finding may be due to the reduction of blood viscosity by the application of the contrast medium. The finding of contrast-induced nephropathy in one patient underlines the importance of monitoring renal function after cardiac catheterization.
Coronary arterial fistulas in childhood
Authors: Wong Kin-tak , Menahem Samuel
Published: August 2008

We reviewed 16 patients with coronary arterial fistulas seen between 1976 and 1997, and aged 2 days to 16 years, with a median age of 3.2 years. Only four patients were symptomatic: two had heart failure, one had exertional dyspnoea, and one infective endocarditis. The fistulas originated from the right coronary artery in seven patients, from the left coronary artery in seven, from both coronary arteries in one patient, while the origin was not clearly defined in the final patient. Associated cardiac anomalies were discovered in six patients, with three of the fistulas being diagnosed at the same presentation. Cross-sectional echocardiography had revealed a dilated coronary artery in 7 out of 11 subjects. The ratio of pulmonary to systemic flows ranged between 0.9 to 3.0, with a median of 1.5. Ten patients were referred for corrective surgery without any mortality. Trans-catheter closure was successfully undertaken in one patient, while spontaneous closure of the fistula was noted in two patients. We conclude that coronary arterial fistulas, although rare and potentially serious, are generally treatable.
Myocardial perfusion scanning in patients considered for late arterial switch
Authors: McLeod Karen A , De Giovanni Joseph , Silove Eric , Alton Helen , Poyner Robert , Brawn William
Published: August 2008

Background

Our aims were to evaluate left ventricular uptake of radionuclide in patients with Mustard's or Senning's procedure, comparing them with patients who had undergone banding of the pulmonary trunk and conversion to the arterial switch.

Methods

Technetium perfusion scans were performed on 27 patients (25 male), aged from 10 to 28 years with a mean of 17.8 years and a standard deviation of 5.8 years, who had undergone Mustard's or Senning's procedure for correction of complete transposition. Of the 27 patients, six had been accepted for staged conversion to an arterial switch. At the time of the study, two of the six patients had undergone completion to the switch and four had undergone banding of the pulmonary trunk with two then proceeding to the arterial switch. Cardiac catheterisation to measure left ventricular pressure was performed in all six patients and scores for left ventricular uptake of isotope were compared with echocardiographic index of the thickness of the left ventricular posterior wall and measure ments of left ventricular pressure.

Results

Uptake of isotope by the left ventricle was generally poor, but was higher in patients following banding and conversion to the arterial switch, as well as in two patients with native obstruction of the left ventricular outflow tract, and one other who subsequently was found to have pulmonary venous obstruction. There was a positive correlation between the thickness of the left posterior wall in diastole and left ventricular uptake of isotope (r = 0.74, p< 0.05). There also a positive correlation between left ventricular pressure and uptake of the isotope (r = 0.68, p<0.05).

Conclusions

Uptake of radionuclide by the left ventricle after Mustard's or Senning's procedure for complete transposition appears to reflect ventricular pressure and myocardial mass. A prospective study would be required to determine the predictive ability of such scans regarding the ultimate outcome of conversion to arterial switch, but our initial findings suggest that the technique provides an additional non-invasive method of monitoring left ventricular response to pulmonary arterial banding.
Neonatal intrapericardial teratomas: clinical and surgical considerations
Authors: Pratt Jerry W. , Cohen Daniel M. , Mutabagani Khaled H. , Davis J. Terrance , Wheller John J.
Published: August 2008

Intrapericardial teratomas are rare primary cardiac tumors of infancy and childhood. We describe three neonates with intrapericardial teratomas diagnosed during fetal life and treated after birth. Clinical and anatomic considerations suggest that cardiopulmonary bypass provides for safe tumor dissection and complete excision of the tumor, thereby decreasing the risk of recurrence.
A unique case of ventricular isomerism?
Authors: Rinne Katy , Smith Audrey , Hot Siew Yen
Published: August 2008

Isomerism of the atriums has often been described with various complex cardiac malformations. As far as is known, a case of ‘ventricular isomerism’ has never been recorded. Described is a specimen where, on the basis of morphological criterions, there are two right ventricles.
Coarctation of the aorta in dizygotic twins
Authors: Caldas Monica , Dhillon Rami
Published: August 2008

The incidence of congenital heart disease is higher in monozygotic than dizygotic twins, with a higher concordance rate. Although coarctation of the aorta has previously been reported in monozygotic twins, to the best of our knowledge it has not been described in dizygotic twins. We report here such a concurrence in dizygotic twins conceived by in-vitro fertilisation. The finding provides support for both genetic and environmental factors in the aetiology of congenital heart disease. Furthermore, it highlights our lack of data regarding the outcome of in-vitro fertilisation.
Sudden cardiac death in infancy due to histiocytoid cardiomyopathy
Authors: Grech Victor , Ellul Bridget , Montalto Simon Attard
Published: August 2008

Detailed post-mortem is crucial in infants who die suddenly and without a known cause. We report a rare case of histiocytoid cardiomyopathy with endocardial fibroelastosis, the second case in the world literature. The infant presented with sudden death, but the cardiac histological appearance was initially believed to be caused by Pompes disease.
A “friendly” system for pericardial drainage
Authors: da Cruz E. , Carbognani D.
Published: August 2008

We describe an alternative to the water-seal system, suitable for drainage of pericardial and eventually pleural effusions, which has been shown to safe, effective, not painful, and rather “friendly”, providing patients with a smoother clinical course.
Fetal diagnosis of common arterial trunk with interrupted aorticarch using color Power Doppler angiography
Authors: Yasukochi Satoshi , Satomi Gengi
Published: August 2008

We successfully visualized the brachiocephalic arteries and aortic arch in a fetus seen at 19 weeks of gestation with a common arterial trunk and interrupted aortic arch by means of color power Doppler angiography, a new diagnostic development of color Doppler echocardiography. Power Doppler imaging is more sensitive to the state of low flow in fetal vessels, thus providing better visualization of fetal vascular structures from an early gestational stage.
Early onset of progressive subaortic stenosis after complete repair of tetralogy of Fallot
Authors: Grech Victor , Mifsud Anton
Published: August 2008

Tetralogy of Fallot is often found in association with a wide variety of other cardiac lesions, but is rarely found in association with lesions causing obstruction to the left ventricular inflow or outflow. Subaortic stenosis has only rarely been reported in association with tetralogy of Fallot. We report a patient with Marfan syndrome who underwent repair of tetralogy of Fallot at five years of age. Discrete and progressive subaortic stenosis developed two years after the surgical correction, in a previously normal and unobstructed left ventricular outflow tract. Surgical removal of the acquired fibrous subaortic shelf was successful. Clinical signs of obstruction within the left ventricular outflow tract after surgical repair of tetralogy of Fallot should prompt further investigation to exclude the onset of acquired subaortic stenosis.
A most peculiar coronary circulation in a patient with pulmonary atresia and intact ventricular septum
Authors: Freedom Robert M. , Yoo Shi-Joon , Javois Alexander
Published: August 2008

A patient with pulmonary atresia and intact ventricular septum was found to have a right ventricular-dependent coronary circulation. In this infant both coronary arteries lacked their normal proximal connection with the aorta, perhaps the most egregious form of this prejudicial coronary circulation. Even more interesting was a direct collateral vessel originating from the descending thoracic aorta and connecting with the coronary circulation. This patient has undergone bilateral modified Blalock-Taussig shunts, and left ventricular function seems preserved.
Anterograde double-balloon valvoplasty for treatment of severe valvar aortic stenosis in a preterm baby weighing 1400 grams
Authors: Peuster Matthias , Paul Thomas , Hausdorf Gerd
Published: August 2008

We describe our treatment of a premature baby born weighing 1400 g with severe aortic stenosis, with a gradient of 80 mmHg across the valve. Efforts to advance a 6 mm angioplasty catheter into the stenotic aortic valve via the left ventricle failed. Anterograde angioplasty, instead, was performed using two 4 mm coronary angioplasty catheters. Six months subsequent to the intervention, the pressure gradient measured 25 mmHg, and there was no hemodynamically significant aortic insufficiency.
Tetralogy of Fallot associated with scimitar syndrome
Authors: Azhari Nawal , Al-Fadley Fadel , Bulbul Ziad R.
Published: August 2008

We present a case of tetralogy of Fallot associated with Scimitar syndrome. The patient was an 11-month old female who underwent successfully total repair of her lesion, including rerouting of the anomalous pulmonary vein to the left atrium. The diagnosis was suspected from the chest x-ray and echocardiography, and confirmed by angiography. To the best of our knowledge only 2 additional cases have previously been reported.
New insight into left ventricular function in tricuspid atresia after total cavopulmonary connection: a three-dimensional echocardiographic study
Authors: Acar Philippe , Saliba Zakhia , Sidi Daniel , Kachaner Jean
Published: August 2008

Background

In patients with tricuspid atresia palliated by construction of a total cavopulmonary connection, both pulmonary and systemic circulations depend on the performance of the dominant left ventricle. When estimating the volume of such ventricles using cross-sectional echocardiography, it is necessary to make assumptions concerning the geometry of the ventricular shape. This is avoided by three-dimensional echocardiography, which provides direct volumetric data. Our purpose was to apply this new method to quantify left ventricular volumes and function in patients with tricuspid atresia after construction of a total cavopulmonary connection.

Methods

We studied ten patients (median age: 8 years) with tricuspid atresia who had undergone a total cavopulmonary connection, comparing them with 10 normal children matched for age, sex and size. The three-dimensional echocardiography was performed with electrocardio graphic and respiratory gating. A new transthoracic integrated probe designed for small children was used with a rotational scanning increment of 3°. The 60 slices obtained from the ventricular cavity were stored and formatted in a commercial system (TomTec®). End-diastolic and end-systolic volumes, stroke volume and ejection fraction were calculated after manual tracing of the endocardial surfaces. The volumes were indexed to the body surface area.

Results

As seen in the reconstructions, the dominant left ventricle in tricuspid atresia had a spherical shape, whereas the normal left ventricle is oblong. The left ventricular volumes and function in tricuspid atresia were 54±4 ml/m2(end-diastolic volume), 28±3 ml/m2(end-systolic volume), 26±7 ml/m2(stroke volume) and 48±6% (ejection fraction). These volumes were not different from those obtained in the controls (p = NS). The left ventricular stroke volume and ejection fraction in 10 patients with tricuspid atresia were lower than those calculated for the controls (p < 0.05).

Conclusions

Three-dimensional echocardiography provides a quantitative insight into the pathophysiologic function of the dominant left ventricle in tricuspid atresia after construction of a total cavopulmonary connection.
Physical activity in children and adolescents with congenital heart disease. Aspects of measurements with an activity monitor
Authors: Fredriksen P. M. , Ingjer E. , Thaulow E.
Published: August 2008

The aim of the present study was to evaluate different aspects of a device designed to monitor physical activity. Measurements of different axes and placement of the monitor were tested using a treadmill with ranging increments in incline or speed. The monitor was also used to assess the level of physical activity among children and adolescents with congenital heart disease and in healthy controls at the same age. The results indicate that the monitor is a valid and reproducible instrument for measurements of physical activity. The study revealed that the level of activity was higher for healthy boys than healthy girls (p<0.0001). Boys with congenital heart disease also displayed higher values compared to girls with congenital heart disease, although the difference was not significant (p=0.067). Healthy boys revealed a significantly higher level of activity than did boys with congenital heart disease (p=0.003), but no such difference was found in girls (p=0.757). Nor were any differences found between younger and older indi viduals among patients with congenital heart disease. Young healthy controls, however, showed significantly higher levels of activity than their older counterparts. There were differences in activity monitored during the week, with lower activity in the weekends, but the activity on the same day in different weeks seemed stable. Neither were there any differences between measurements over whole weeks. The results indicate that the Computer Science & Application monitor is a valid instrument for assessing physical activity. The monitor may also be used, therefore, to validate the levels of physical activity level in children with congenital heart disease after medical and surgical treatment.
Effect of physical training in children and adolescents with congenital heart disease
Authors: Fredriksen Per Morten , Kahrs N. , Blaasvaer S. , Sigurdsen E. , Gundersen O. , Roeksund O. , Norgaand G. , Vik J.T. , Soerbye O. , Ingjer F. , Thaulow E.
Published: August 2008

In order to test the effect of systematic supervised physical training, we divided a total of 129 children and adolescents with congenital heart disease into a group undergoing intervention and a control group. All patients underwent exercise tests, measurements of physical activity, and a survey of psychosocial factors. An improvement in uptake of peak level of oxygen was observed after intervention. There was also an improvement in physical activity in both groups measured by a monitor, although this was significant only in those with intervention. The psychosocial scales measured by the Child Behavior Checklist showed a decrease in internalizing scores for those subjected to intervention. This was decreased due to decreased withdrawal and somatic complaints. In conclusion, we recommend systematic supervised training, including testing of routine follow-ups, in patients with congenital heart disease.
Non-invasive determination of the systolic peak-to-peak gradient in children with aortic stenosis: validation of a mathematical model
Authors: Lima Valter C. , Zahn Evan , Houde Christine , Smallhorn Jeffrey , Freedom Robert M. , Benson Lee N.
Published: August 2008

Doppler derived systolic pressure gradients have become widely applied as noninvasively obtained estimates of the severity of aortic valvar stenosis. There is little correlation, however, between the Doppler derived peak instantaneous gradient and the peak-to-peak gradient obtained at catheterisation, the latter being the most applied variable to determine severity in children. The purpose of this study was to validate a mathematical model based on data from catheterisation which estimates the peak-to-peak gradient from variables which can be obtained by noninvasive means (Doppler derived mean gradient and pulse pressure), according to the formula: peak-to-peak systolic gradient=6.02+1.49*(mean gradient)−0.44*(pulse pressure). Simultaneous cardiac catheterization and Doppler studies were performed on 10 patients with congenital aortic valvar stenosis. Correlations between the gradients measured at catheter measured, and those derived by Doppler, were performed using linear regression analysis. The mean gradients correlated well (y=0.67 × + 11.11, r=0.87, SEE=6 mm Hg, p=0.001). The gradients predicted by the formula also correlated well with the peak-to-peak gradients measured at catheter (y=0.66 × + 14.44, r=0.84, SEE=9 mm Hg, p=O.002). The data support the application of the model, allowing noninvasive prediction of the peak-to-peak gradient across the aortic valvar stenosis.