1/43. Sudden death due to ventricular septal defect.Ventricular septal defects (VSD) are usually considered non-life-threatening, usually closing spontaneously or causing symptoms of congestive heart failure, which can be surgically treated in time to save the patient's life. Despite the usually benign clinical course of VSD, serious arrhythmias occur in 16-31% of patients. Sudden death accounted for one-third of all deaths in a series of medically managed patients and occurred in 4.2% of patients in a study of VSD and arrhythmias. Cardiac hypertrophy is the common denominator in all cases reported in detail of VSD-associated sudden death. We have encountered four cases of sudden unexpected death from VSD in infants ranging in age from 1 week to 3 and (1/2) months. In each case there was cardiomegaly and in one case there was pulmonary arteriolar medial thickening, with extension of smooth muscle into small intralobular vessels. In half of our cases the attending physician was sued for malpractice. We believe that VSD in infants and young children are potentially life-threatening malformations which warrant careful clinical follow-up.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/43. Stenting of stenosed aortopulmonary collaterals and shunts for palliation of pulmonary atresia/ventricular septal defect.patients with unrepaired pulmonary artery atresia and ventricular septal defect (PA/VSD) depend on aortoplumonary collaterals and surgically created shunts for pulmonary blood flow. These vessels frequently develop stenoses with time, leading to hypoperfusion of lung segments and systemic hypoxemia. The purpose of this article is to describe catheter palliation of hypoxemic patients with PA/VSD who were not candidates for surgical repair. We present our experience with stent implantation for stenosis of aortopulmonary collaterals and shunts in these patients. Three patients with hypoplastic pulmonary arteries underwent stent placement in aortopulmonary collateral arteries (APCAs) or their shunts. Technical aspects of the interventional catheterization procedure are discussed in detail. Case 1 underwent placement of five stents in collateral vessels and one stent in the Blalock-Taussig shunt (BT) with dramatic increase in vessel size and improvement in saturations from 70% to 89%. Case 2 underwent placement of two overlapping stents in a collateral vessel with an increase in diameter of the collateral vessel from 2.3 to 6 mm and an improvement in saturation from 68% to 88%. Case 3 underwent placement of three overlapping stents in a BT shunt with an increase in diameter of the shunt from 2.2 to 6.6 mm and an improvement in saturation from 71% to 89%. All three patients had excellent clinical improvement and stable saturation at follow-up. Stent placement for maintaining patency of APCAs and aortopulmonary shunts is feasible and safe.- - - - - - - - - - ranking = 5keywords = vessel (Clic here for more details about this article) |
3/43. Right juxtaposition of the atrial appendages.We present an infant with right-sided juxtaposition of atrial appendages who had open heart surgery for ventricular septal defect and patent ductus arteriosus. Of 12 cases thus far reported, ventricular d-loop was observed in nine, and normal position of great vessels in four. Contrary to previous views, this condition may not be accompanied by severe conotruncal anomalies.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/43. Rhabdomyomatous dysplasia of the newborn lung associated with multiple congenital malformations of the heart and great vessels.Interstitial proliferation of striated muscle in the lung is extremely rare. Most cases are associated with other congenital malformations, such as lung sequestration, diaphragmatic hernia, or cardiac malformations. We describe a newborn with rhabdomyomatous dysplasia of the lung associated with multiple congenital malformations of the heart and great vessels. The female neonate was born at 37 weeks of gestation as the second child to a 31-year-old woman without relevant previous medical or family history. In week 26 of gestation, a complex heart malformation and polyhydramnion were diagnosed by ultrasound. Postnatally, right lung hypoplasia, a bilobar right and left lung, anomalous drainage of the pulmonary veins, atrial and ventricular septal defects, and double-outlet right-ventricle and multiple aortopulmonary collaterals were described. Histological examination of a biopsy of the right lung demonstrated the presence of numerous bundles of striated muscle fibers arranged randomly in the pulmonary interstitium. Unilateral resection of the right lung was not a therapeutic option, because the left lung had developed bronchopulmonary dysplasia with severe reduction in gas exchange as a consequence of long-term mechanical ventilation. Symptomatic relief and palliative cardiac surgery were offered. At age 5 months, the infant died of a pulmonary hemorrhage following cardiac surgery.- - - - - - - - - - ranking = 5keywords = vessel (Clic here for more details about this article) |
5/43. An unusual form of the transposition complex. Uncorrected levo-transposition with horizontal ventricular septum: Report of two cases.A distinctive angiographic appearance is described in two patients who had uncorrected levo-transposition of the great vessels. Although levo-transposition with inversion of the ventricles usually results in physiologically corrected transposition, in these patients the anatomy was arranged in such a way as to result in an uncorrected transposition. The following elements were responsible for this physiological condition: normal atrial situs, inverted ventricles with "criss-cross" atrioventricular flow, levo-transposed great arteries. In addition, the morphological right ventricle was hypoplastic, left sided and superior to the left ventricle and the ventricular septum was horizontal in position. The two ventricles were connected via a large ventricular septal defect. The importance of accurate, detailed, preoperative angiographic demonstration of the anatomic situation is stressed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/43. Successful closure of ventricular septal defect through a left-sided ventriculotomy in corrected transposition of the great vessels.Corrected transposition of the great vessels is often associated with other cardiac anomalies. Ventricular septal defect (VSD) is the most common among them. Closure of a VSD is usually performed through a right-sided ventriculotomy. We had previously closed the VSD through the right-sided ventricle in 2 patients with corrected transposition of the great vessels and in 2 others with double-outlet right ventricle with ventricular inversion, which resulted in complete heart block. Recently we have closed the VSD through the left-sided ventricle in 3 patients with corrected transposition, and no block resulted.- - - - - - - - - - ranking = 6keywords = vessel (Clic here for more details about this article) |
7/43. Isolated noncompaction of myocardium associated with calcification in the interventricular septum.We describe a 12-year-old male with isolated noncompaction of the myocardium and associated abnormal calcification in the basal interventricular septum, and we present a review of the literature. The patient has been healthy and free of symptoms. The electrocardiogram showed abnormal Q waves in III, V1, V2, and ST elevation in V1-V3. exercise testing demonstrated ST depression in V4 and V5. Myocardial scintigraphic examination showed a regional reduction in iodine-1,2,3-beta-methyl-iodophenylpentadecanoic acid uptake in the basal interventricular septum. Since coronary angiography demonstrated normal coronary vessels and the trabeculations were not prominent in this region, we hypothesize that coronary microcirculatory dysfunction may cause subendocardial infarction associated with calcification in the same area.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/43. aneurysm of right-sided cervical arch: surgical removal and graft replacement.Right-sided cervial aortic arch is a rare congenital anomaly which may cause respiratory symptoms or dysphagia. In the past, surgical correction of the cervical arch has not been attempted except in one patient in whom an erroeous diagnosis of aneurysm of the innominate artery led to an unsuccessful operation. A case is reported of a 39-year-old woman with an extensive fusiform aneurysm of a right-sided cervical arch. The arch aneurysm was successfully removed surgically and normal circulation was restored to the arch vessels. At the same operation, a ventricular septal defect was repaired with the aid of temporary cardiopulmonary bypass.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/43. Conversion of lusoric artery into right subclavian artery in one-stage neonatal repair of aortic arch anomalies and intracardiac defects.In 2 patients, a lusoric artery was compressing the mediastinal structures during a one-stage repair of type B aortic arch interruption and ventricular septal defect. In order to prevent the long-term complications of sacrifice of the subclavian artery, the lusoric artery was not simply divided but converted into a regular right subclavian artery by re-implantation of the abberant vessel at the origin of the right carotid artery during the one-stage repair. At follow up, both patients have normal right radial arterial pulsations. In one patient, angiography was repeated and confirmed a well functioning right subclavian artery. Conversion of a lusoric artery into a right subclavian artery during one-stage repair of aortic arch anomalies and intracardiac defects is not only feasible but also indicated to preserve subclavian artery function and to prevent compression of the mediastinal structures by a vascular ring.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/43. Pathological lesions causing pulmonary hypertension after closure of a ventricular septal defect.A 15-year-old boy with a ventricular septal defect, pulmonary hypertension, Down's syndrome, and extremely thickened media (ETM) of the small pulmonary arteries died of heart failure and pulmonary hypertension 13 years after intracardiac repair. Microscopic examination of lung specimens collected prior to the intracardiac repair and at the time of autopsy revealed that the ETM had remained unchanged and that the arteries connected to the vessels with ETM had become severely thickened. The present case shows that even a small percentage of arteries with ETM can cause pulmonary hypertension, and illustrates one of the mechanisms of how pulmonary hypertension can fail to be resolved after intracardiac repair.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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