Complications of congenital portosystemic shunts in children: therapeutic options and outcomes.

BACKGROUND AND OBJECTIVE: Congenital portosystemic shunts are rare vascular malformations that lead to severe complications. Their management is controversial. The aim of this study was to propose a clear definition of the risks and management of congenital portosystemic shunts in children according to our experience and a review of the literature. PATIENTS AND METHODS: Twenty-two children with a complicated congenital portosystemic shunt were studied in our institution. When necessary, management included portal pressure measurement and portal vein angiography during an occlusion test and closure of the shunt by surgical and/or endovascular methods. RESULTS: Five neonates with intrahepatic shunts presented with cholestasis that resolved spontaneously, and 17 older children presented with liver tumors (13) and/or hepatopulmonary syndrome (2), pulmonary artery hypertension (3), portosystemic encephalopathy (3), heart failure (1), and glomerulonephritis (1). The portosystemic shunt was extrahepatic (11) or intrahepatic (6). Portosystemic shunts were closed by endovascular methods in 5 children and surgically in 10, 4 of whom had portal pressure during occlusion above 35 mmHg and extremely hypoplastic or undetectable portal veins requiring banding of the fistula before closure. Shunt closure resulted in restoration of intrahepatic portal flow in all, with complete or partial regression of benign liver masses, and regression or stabilization of pulmonary, cardiac, neurological, and renal complications. CONCLUSIONS: Congenital portosystemic shunt carries risks of severe complications in children. Closure of a shunt persisting after age 2 years should be considered preventively. Intrahepatic portal flux restoration can be expected, even when intrahepatic portal veins are extremely hypoplastic or undetectable.

Mean pulmonary artery pressure estimated from systolic and diastolic pulmonary artery pressure in children with congenital heart disease: an invasive study.

This hemodynamic study documented the accuracy and precision of six empirical formulas relating mean (mPAP), systolic (sPAP), and diastolic (dPAP) pulmonary artery pressures in children with congenital heart disease. Fluid-filled PAPs of 61 children (age, 26 +/- 40 months) were analyzed over an mPAP range of 7 to 74 mmHg. All formulas were accurate (mean bias, -2 to 1 mmHg). The three formulas relying on sPAP and dPAP were dPAP + 1/3 (sPAP - dPAP), dPAP + 0.41 (sPAP - dPAP), and sqrt of (sPAP x dPAP). They were precise (bias standard deviation [SD], 3 mmHg), with approximately 90% of patients exhibiting biases of less than 5 mmHg. The three formulas relying on sPAP alone all assumed that mPAP approached two-thirds of sPAP according to slightly different mathematical equations. They were less precise (bias SD, 5-6 mmHg), with biases greater than 7 mmHg for 10% to 16% patients. Accurate estimates of mPAP were obtained from sPAP alone, and this could be valuable for cross-checking the self-consistency of the pressure database obtained in the echo-Doppler laboratory. For cases that had reliable dPAP estimates available, empirical formulas relying on both sPAP and dPAP were more precise and thus must be preferred.

Conotruncal defects associated with anomalous pulmonary venous connections.

BACKGROUND: Conotruncal defects constitute one of the major categories of congenital heart disease. Our understanding of how these defects develop has been derived from knowledge of the role of neural crest cells in heart development. However, recent studies have revealed a role for the myocardium in the formation of both the arterial and venous poles of the heart. AIM: To identify congenital heart defects that associate anomalies of the arterial and venous poles. METHODS: From a database spanning 27 years, we identified those patients with conotruncal defects associated with an anomalous pulmonary venous connection (APVC; total or partial). Patients with atria isomerism or atrioventricular septal defects were excluded. Patient files were reviewed for clinical presentation, family history, diagnostic and surgical procedures, and outcome. RESULTS: We identified 23 patients with conotruncal defects and APVC. Conotruncal defects were as follows : common arterial trunk, n=7; tetralogy of Fallot, n=5; discordant ventriculoarterial connections, n=4; interrupted aortic arch, n=2; subarterial ventricular septal defect, n=2; double outlet right ventricle, n=2; and right pulmonary artery from ascending aorta, n=1. Nine patients had total APVC and 14 patients had partial APVC. Recurrence of the cardiac defects in siblings was observed in three families. CONCLUSION: Our findings suggest that congenital heart defects that associate anomalies of the arterial and venous poles may have a common embryology, which results from a myocardial defect.

Efficiency of transcatheter closure of atrial septal defects in small and symptomatic children.

OBJECTIVES: We report the multicentric French experience with transcatheter closure in children weighing 15 kilograms or less, with the aim of assessing the efficacy of the procedure in this age group. PATIENTS: We included all children weighing 15 kilograms or less, and seen between January, 1997, and June, 2004, who had successful transcatheter closure of an interatrial communication within the oval fossa. RESULTS: Transcatheter closure was performed in 35 patients weighing 15 kilograms or less, of whom 14 were male and 21 female. The procedures were undertaken in 8 different centres, the patients having a median age of 3 years, with a range from zero to 6.2 years, and a mean weight of 13 kilograms, with a range from 3.6 to 15 kilograms. All the patients were symptomatic, with associated cardiac malformations present in 4 cases, and extracardiac anomalies in 4 patients, including Down's syndrome in 3, and Adams Oliver syndrome in the other case. In 1 patient, emergency cardiac surgery was needed 24 h after the procedure to correct a previously undiagnosed divided right atrium. No other complication occurred. After a median follow-up of 2 years, with a range from 0.5 to 5.2 years, all the patients are asymptomatic, except for one long-standing patient with bronchodysplasia. In 1 other patient, a small residual bidirectional shunt was detected by echocardiography. No patient presented significant arrhythmia. In the patients followed-up for more than 12 months, we found a significant gain in weight gain. CONCLUSION: Transcatheter closure of an interatrial communication within the oval fossa is efficient in children weighing 15 kilograms or less, and can be proposed as a first line of treatment in symptomatic patients. Children with retarded growth tend to have complete recovery within one year of closure.

Aortic valve regurgitation after arterial switch operation for transposition of the great arteries: incidence, risk factors, and outcome.

OBJECTIVES: The aims of this study were to assess the prevalence and incidence of aortic valve regurgitation (AR) after arterial switch operation (ASO), its outcome, and the risk factors. BACKGROUND: After an ASO, the long-term fate of the aortic valve is a concern as follow-up lengthens. METHODS: Operative and follow-up data on 1,156 hospital survivors after ASOs between 1982 and December 2000 were reviewed. RESULTS: At last follow-up (mean duration 76.2 +/- 60.5 months), 172 patients (14.9%) had an AR. Complex transposition of the great arteries, prior pulmonary banding done in 75 patients (21 with intact ventricular septum), aortic arch anomalies, AR at discharge, older age at ASO, and aortic/pulmonary size discrepancy were associated with AR. On multivariate analysis, the presence of a ventricular septal defect (VSD) or AR at discharge multiplied the risk by 2 and 4, respectively. Freedom from AR was 77.9% and 69.5% at 10 and 15 years, respectively; hazard function for AR declined rapidly and slowly increased thereafter. Reoperation from AR was done in 16 patients with one death, valvuloplasty being unsuccessful. Freedom from reoperation for AR was 97.7% and 96.8% at 10 and 15 years, respectively; hazard function slowly increased from 2 to 16 years. Higher late mortality was not associated with AR. CONCLUSIONS: After ASO, AR was observed and was related to VSD with attending high pressure and flow and AR at discharge. Progression of AR was slow, but incidence increased with follow-up. Reoperation for AR was rare. Late aortic valve function warrants long-term monitoring.

Transcatheter occlusion of an intrahepatic venovenous fistula after modified Fontan circulation by implantation of an Amplatzer atrial septal occluder.

We report two successful transcatheter closures of intrahepatic venovenous malformations observed after bidirectional cavopulmonary anastomoses. These malformations were detected immediately after surgery in one case and during a 10-year follow-up in the second case. These intrahepatic venovenous malformations were successfully occluded by the implantation of an Amplatzer atrial septal occluder.

[Percutaneous closure of the inter-auricular communications, well defined indications]. / Fermeture percutanée des communications inter-auriculaires, des indications bien définies.

POSSIBLITIES FOR PROGNOSTIC IMPROVEMENT: The inter-auricular communication (IAC) is the most frequent of congential malformations in adults and is responsible for enhanced morbidity and rduction in life expectancy. The closure of an IAC with volumic overload eradicates such morbidity and mortality. The technical progress in the conception of prosthesises that obstrue the interauricular communications via the percutaneous route make this technique a reliable alternative to surgery that, itself, leads to a certain degree of discomfort and considerable morbidity. DEPENDING ON THE ECHOCARDIOGRAPHICAL DATA: The selection of patients is crucial and relies on trans-thoracic echocardiography. It is indicated in isolated ostium secundum-type inter-auricular communications with a border of at least 4 mm separating it from the adjacent structures of the heart; the size on the echocardiography must not exceed 30 mm. FROM A TECHNICAL POINT OF VIEW: The closure is made under fluoroscopic and echocardiographic control via the trans-oesophageal route. It is rapid, simple, and induces a low rate of morbidity. With Amplatz's prosthesis, the percentage of complete closure is equivalent to that obtained with surgery but with less morbidity with regard to arrhythmia. Today the reliable percutaneous closure of an IAC will probably permit the extension of the indications for the closure of this congenital abnormality in adults.

Left-sided lesions after anatomic repair of transposition of the great arteries, ventricular septal defect, and coarctation: surgical factors.

OBJECTIVE: This study was undertaken to identify potential anatomic and surgical factors creating left-sided lesions, namely recoarctation of the aorta and neoaortic regurgitation, after anatomic repair of transposition of the great arteries with ventricular septal defect and aortic coarctation. METHODS: From 1983 to September 2002, 109 survivors out of 120 patients were studied. Two-stage repair was performed in 42 patients (group A), and single-stage repair was performed in 67 (groups B and C). Before repair, the diameters of the ascending aorta and main pulmonary artery were measured. In the patients with single-stage repair, coarctation was repaired by extended end-to-end anastomosis in 35 patients (group B) and by pulmonary homograft patch augmentation in 32 patients (group C). The ventricular septal defect was closed through the pulmonary artery in 70 patients and through the right ventricle or atrium in 39 patients. The neoaorto-aortic discrepancy was treated by V-shaped resection of the posterior sinus of Valsalva in 7 cases, pulmonary homograft patch in 32 cases, and anterior splitting of the ascending aorta in all cases. Before discharge from the hospital, neoaortic root and ascending aorta diameters and aortic regurgitation grade were recorded. Neoaortic regurgitation progression and reintervention were the end points of follow-up (97.2 +/- 61.2 months). RESULTS: Early and late survivals were significantly better in group C (P <.001). Risk factors for neoaortic regurgitation at discharge by univariate analysis were single-stage repair (P <.05) and ventricular septal defect closure through the pulmonary artery (P =.0076). On multivariate analysis, the latter was the only risk factor for neoaortic regurgitation at discharge and at last follow-up. Multivariate analysis showed that higher neoaortic root/ascending aorta ratio and ventricular septal defect closure through the pulmonary artery were risk factors for neoaortic regurgitation evolution at last follow-up. There were 29 reinterventions, 19 for recoarctation of the aorta and 10 for neoaortic regurgitation with or without aortic root dilatation. Group B (P <.05), high neoaortic root/ascending aorta ratio (P <.01), and progressive neoaortic regurgitation (P <.05) were risk factors for recoarctation of the aorta. Group A was a risk factor for aortic valve replacement at 10 years (P <.05). CONCLUSION: Neonatal single-stage repair with pulmonary homograft aortic augmentation remains the optimal approach to transposition of the great arteries with ventricular septal defect and aortic coarctation. It provides better early and late survivals and freedoms from left-sided lesions. Avoidance of late recoarctation of the aorta and progressive neoaortic regurgitation requires meticulous closure of the ventricular septal defect and evenly sized reconstruction of the aorta from root to distal arch.