Infantile scimitar syndrome with severe pulmonary hypertension: successful treatment with coil embolization of the systemic arterial supply to the sequestered lung

Scimitar syndrome is a rare cardiopulmonary malformation. Presentation in infancy is usually associated with pulmonary hypertension and severe symptoms. We discuss treatment of such an infant. Two abnormal systemic vessels supplying the sequestered lower part of the right lung were embolized using catheter-inserted coils. The patent arterial duct was surgically ligated. These procedures resulted in a significant reduction of the shunt and the level of pulmonary hypertension, as well as in an impresssive improvement of symptom

Transcatheter embolization of abnormal intrathoracic vessels using coils in the setting of children with congenital heart disease

Abstract Objective it was the purpose of this retrospective study to assess the efficacy and the rate of complications of transcatheter embolization of abnormal intrathoracic vessels using coils in children with complex congenital heart disease. Patients and methods in 17 children (mean age 9.2±5.9 years) with complex congenital heart disease, occlusion by catheter intervention was attempted in 29 abnormal intrathoracic vessels: 13 aorto-pulmonary collaterals, 12 arterio-pulmonary collaterals, 2 systemic arteries supplying pulmonary sequestrations, one central venous connection and a Blalock-Taussig shunt. The mean diameter of the vessels was 4.7 ± 1.6 mm (range 2-8 mm). Steel coils with a helical diameter of three, five or eight mm were used. After selective catheterization of the vessel, they were delivered through a 5 F endhole catheter. The helical diameter of the coils was chosen in order to exceed the inner diameter of the vessel by 10-30%. Results as assessed by selective angiography performed 10 minutes or more after release of the coil, 27 of the 29 vessels (93%) were successfully occluded. A mean of 2.6 coils (range 1-11) were necessary for successful occlusion. Complications were encountered during 4 attempts of occlusion (14%). Of a total of 76 coils delivered, 4 coils (5%) secondarily migrated after release from the catheter, mostly to branches of the pulmonary arteries. This complication was seen predominantly in those vessels with the largest diameters. Three of the dislocated coils were left in place as they did not significantly obstruct flow of blood. One coil was retrieved by a basket catheter. Conclusion transcatheter embolization of abnormal intrathoracic vessels using coils in children with congenital heart disease is an effective therapy. Potential complications warrant careful evaluation of the indications for these procedure

Cardiomegaly in a premature neonate after venous umbilical catheterization

Umbilical venous catheters allow rapid central access in neonates, but may be associated with various complications. We present a case of a newborn with pericardial effusion following umbilical venous catheterization. An extremely low birth weight infant was intubated for respiratory distress syndrome and had umbilical venous and arterial lines in place. Massive cardiomegaly was noted on the subsequent chest X-ray. Echocardiography revealed a large pericardial effusion without signs of tamponade. After removing the catheter, the effusion gradually resolved. While pericardial effusion is a well-known complication of percutaneous long central lines, only a few case reports have documented sudden cardiovascular compromise associated with umbilical venous catheters. Pericardial effusion may be asymptomatic and should be suspected in infants with central catheters and progressive cardiomegaly. The prompt removal of catheters and, if signs of cardiac tamponade are present, emergency pericardiocentesis may prove to be life-savin

Surgery of the dilated aortic root and ascending aorta in pediatric patients: techniques and results

Objective: Dilatation of the aortic root is a well-known cardiovascular manifestation in children and adult patients with connective tissue disease (e.g. Marfan syndrome). Dilatation of the ascending aorta is extremely rare and may be associated with bicuspid aortic valve. This report evaluates the incidence of dilatative aortic root and ascending aortic pathology in patients younger than 18 years and analyzes the results obtained after repair and replacement strategies. Methods: Between 1/1995 and 12/2002, a total of 752 operations on the thoracic aorta were performed in adult and pediatric patients. We present our experience with a group of 26 patients <18 years of age, who required isolated surgery of the aortic root and/or ascending aorta because of a dilatative lesion. Fifteen patients had isolated aortic root dilatation (13 of them suffered from Marfan syndrome), eight patients presented with an idiopathic dilatation of the ascending aorta and three patients had dilatation in association with a bicuspid aortic valve. Mean age was 10±4.8 years (4-18 years). Repair of the aortic root with preservation of the aortic valve (Yacoub, David or selective sinus repair) was performed in nine patients, replacement using a homograft was performed in five patients, composite graft with mechanical prosthesis in two patients, with biological prosthesis in one patient and Ross operation was performed in one case. Isolated supracoronary graft replacement was performed in eight patients. Results: Two patients died during hospitalization: a 10-year old girl developed respiratory failure on the 2nd postoperative day and autopsy revealed Ehlers-Danlos syndrome with a massive intrapulmonary emphysema. A 14-year-old Marfan patient with severely depressed preoperative LV function died from low cardiac output following composite-graft, mitral and tricuspid valve repair. One patient required aortic valve replacement 7 days after an aortic valve sparing root repair. There was no additional perioperative morbidity. In the long-term, two patients died from rupture of the thoracic aorta, both following minor non-cardiovascular surgical procedures. Both had normal sized descending and abdominal aorta. Conclusion: Repair of the aortic root and/or ascending aorta in children and adolescent patients can be performed with acceptable early and late results. While the presence of severe comorbidity may adversely affect early outcome, long-term survival was mainly determined by rupture of the descending aort

Considerations for Prenatal Counselling of Patients with Cardiac Rhabdomyomas based on their Cardiac and Neurologic Outcomes

Cardiac rhabdomyomas are benign cardiac tumours with few cardiac complications, but with a known association to tuberous sclerosis that affects the neurologic outcome of the patients. We have analysed the long-term cardiac and neurological outcomes of patients with cardiac rhabdomyomas in order to allow comprehensive prenatal counselling, basing our findings on the records of all patients seen prenatally and postnatally with an echocardiographic diagnosis of cardiac rhabdomyoma encountered from August, 1982, to September, 2007. We analysed factors such as the number and the location of the tumours to establish their association with a diagnosis of tuberous sclerosis, predicting the cardiac and neurologic outcomes for the patients. Cardiac complications include arrhythmias, obstruction of the ventricular outflow tracts, and secondary cardiogenic shock. Arrhythmias were encountered most often during the neonatal period, with supraventricular tachycardia being the commonest rhythm disturbance identified. No specific dimension or location of the cardiac rhabdomyomas predicted the disturbances of rhythm. The importance of the diagnosis of tuberous sclerosis is exemplified by the neurodevelopmental complications, with four-fifths of the patients showing epilepsy, and two-thirds having delayed development. The presence of multiple cardiac tumours suggested a higher risk of being affected by tuberous sclerosis. The tumours generally regress after birth, and cardiac-related problems are rare after the perinatal period. Tuberous sclerosis and the associated neurodevelopmental complications dominate the clinical picture, and should form an important aspect of the prenatal counselling of parent

Dimensions of the Ascending Aorta in Conotruncal Heart Defects

Dilatation of the ascending aorta is an important sequel in conotruncal anomalies, such as tetralogy of Fallot (TOF) or d-transposition of the great arteries (TGA). We measured dimensions and their progression at different levels of the ascending aorta in 80 patients. In TOF patients, mean z-score for aortic annulus was 1.65 (range −3.16-6.47), for sinus 1.93 (range −2.28-5.39), for st-junction 4.15 (range 0.0-8.18), and for ascending aorta 3.51 (range −1.23-6.36). Over time, annulus z-scores increased in the univariate analysis [0.07/year, 95% confidence interval (CI) 0.01-0.14; p=0.02], and this was unique to male patients (0.08/year, 95% CI 0.00-0.15; p=0.05). z-scores of the ascending aorta decreased (−0.1/year, 95% CI −0.18 to −0.02; p=0.02), and this was confined to patients without aortic regurgitation (AR; −0.09/year, 95% CI −0.18 to −0.01; p=0.04). In TGA, mean z-score for the aortic annulus was 2.13 (range −3.71-8.39), for sinus 1.77 (range −3.04-6.69), for st-junction 1.01 (range −5.44-6.71), and for ascending aorta 0.82 (range −4.91-6.46). In bivariate analysis, annulus z-scores decreased in females (−0.14/year, 95% CI −0.25 to −0.03; p=0.01) and in patients without AR (−0.07/year, 95% CI −0.14-0.0; p=0.03). z-scores of the ascending aorta increased significantly in males (0.08/year, 95% CI 0.0 to 0.16; p=0.05) and in patients with AR (0.12/year, 95% CI 0.03-0.21; p=0.01). In conclusion, TOF and TGA z-scores of the ascending aorta differ significantly from those of the normal population. Progression of z-scores over time is influenced by diagnosis, sex, and presence of AR

Persistent Left Superior Vena Cava in Cardiac Congenital Surgery

Persistent left superior vena cava (LSVC) is a relatively frequent finding in congenital cardiac malformation. The scope of the study was to analyze the timing of diagnosis of persistent LSVC, the timing of diagnosis of associated anomalies of the coronary sinus, and the global impact on morbidity and mortality of persistent LSVC in children with congenital heart disease after cardiac surgery. Retrospective analysis of a cohort of children after cardiac surgery on bypass for congenital heart disease. Three hundred seventy-one patients were included in the study, and their median age was 2.75years (IQR 0.65-6.63). Forty-seven children had persistent LSVC (12.7%), and persistent LSVC was identified on echocardiography before surgery in 39 patients (83%). In three patients (6.4%) with persistent LSVC, significant inflow obstruction of the left ventricle developed after surgery leading to low output syndrome or secondary pulmonary hypertension. In eight patients (17%), persistent LSVC was associated with a partially or completely unroofed coronary sinus and in two cases (4%) with coronary sinus ostial atresia. Duration of mechanical ventilation was significantly shorter in the control group (1.2 vs. 3.0days, p=0.04), whereas length of stay in intensive care did not differ. Mortality was also significantly lower in the control group (2.5 vs. 10.6%, p=0.004). The results of study show that persistent LSVC in association with congenital cardiac malformation increases the risk of mortality in children with cardiac surgery on cardiopulmonary bypass. Recognition of a persistent LSVC and its associated anomalies is mandatory to avoid complications during or after cardiac surgery