Postoperative graft thrombosis in Fontan procedure.

Fontan repair could be used as the definitive palliation in many forms of complex cyanotic congenital heart disease. But thrombosis can occur after a modified Fontan operation (right atrium-right ventricular connection with a conduit). Appropriate management of this complication includes thrombolytic therapy, thrombectomy and revision (if surgically remediable causes of the thrombosis are identified) or redo operation. Two repair operations were performed for the treatment of thrombosis of the right side of the heart in patients in whom we had previously performed Bjork modification (right atrium-right ventricular connection with a conduit). The thromboses occurred 6 and 9 years after the operation, respectively. In both cases, the redo Fontan operation was successfully performed using a dacron tube graft. Patients were anticoagulated after the operation. Risk of thrombosis of the right side of the heart after the Fontan repair may be minimized with the use of prophylactic anticoagulation in high-risk patients soon after the operation.

A case with Weaver syndrome operated for congenital cardiac defect.

An 11-month-old Turkish female infant with Weaver syndrome together with atrial septal defect and patent ductus arteriosus which was operated successfully is reported. Weaver syndrome is a very rare disorder of unknown etiology characterized by accelerated growth of prenatal onset, advanced osseous maturation, special craniofacial features, umbilical hernia, and hoarse low-pitched cry. Congenital cardiac defect is not a usual finding. The presented case is the first reported child with Weaver syndrome in the literature operated for a congenital cardiac defect.

A case of truncus arteriosus type II.

A case of truncus arteriosus type II is reported. Truncus arterious is an uncommon congenital cardiac defect where a single great vessel exits the heart. Truncus arteriosus is usually fatal, if untreated. This defect occurs when the conus arteriosus and the truncus divide erroneously in the embryo. Palliative surgery in truncus arteriosus has been unsuccessful. Pulmonary banding has been tried and was ineffective and usually fatal. We operated on a nine-month-old (6200 g) male infant with a type II (Edwards-Collett) defect and a large ventricular septal defect. The pulmonary artery average pressure was 51 mmHg. We performed a cardiopulmonary bypass in the usual manner. Pulmonary arteries were resected from the truncal root, and primary end-to-end anastomosis of the truncal root to the ascending aorta was performed. Right ventricle to pulmonary artery continuity was provided using a valveless Gore-Tex graft. We lost our patient due to intractable pulmonary hypertension on the first postoperative day.

Bicuspid aortic valve and coarctation of aorta.

Bicuspid aortic valve (BAV) and coarctation of aorta (COA) are frequently seen together. It is believed that these malformations result from a single developmental diathesis. A case is presented of COA, aortic stenosis and aneurysm of the ascending aorta corrected by patch aortoplasty and commisurotomy. An aneurysm at the site of the coarctation repair can develop as late as 20 to 25 years after surgery. Almost all of the aneurysms described have occurred in patients undergoing patch aortoplasty. We do not recommend this technique except in special cases.

Posterior deviation of left ventricular outflow tract septal components without ventricular septal defect.

OBJECTIVE: To describe 11 patients with narrowing of the left ventricular outflow tract caused by angular posterior deviation of both the outlet septum and the upper part of trabecular septum, which was diagnosed by cross sectional echocardiography in all and confirmed by angiocardiography in seven. RESULTS: Four patients had a subaortic systolic pressure gradient ranging from 23 to 70 mm Hg by Doppler echocardiography; cardiac catheterisation showed a significant (60 and 104 mm Hg) systolic pressure gradient in two. In four cases aortic regurgitation and two tricuspid pouches were shown by Doppler echocardiography, angiocardiography, or both. Four cases had a ridge at the angulation point on echocardiographic examination. Three patients were operated on for systolic pressure gradients of the left ventricular outflow tract and one for severe aortic regurgitation. There was proliferation of collagen-rich fibrous tissue in the subendocardial region on histopathological examination of the myectomy material. A ventricular septal defect had been diagnosed previously by contrast echocardiography in one patient; thus ventricular septal defects may close spontaneously over a period of time including fetal life. A subaortic ridge was detected in one patient at follow up. CONCLUSIONS: Deviation of the outlet and trabecular septa should be considered as a cause of ventricular outflow tract obstruction even when no ventricular septal defect is present.

Incidence and severity of arrhythmias and conduction disturbance after repair of tetralogy of Fallot.

Ventricular and supraventricular arrhythmias and conduction disturbances were evaluated by routine electrocardiography and 24-hour ambulatory monitoring in 31 patients who underwent correction of tetralogy of Fallot. The interval from operation to the study was 1 month to 14 years (mean 4.8 +/- 2.8). Complete right bundle branch block occurred in 22 (71%) patients and incomplete right bundle branch block in 9 (29%) patients. Bifascicular block with right bundle branch block and left axis deviation (LAD) occurred in one patient. Two patients had second degree type II atrioventricular block. Twenty-four-hour ambulatory electrocardiographic monitoring was performed in all patients and they were divided in two groups according to the frequency of ventricular arrhythmias (Lown classification). Group 1 included the 23 patients who had no arrhythmia or rare ventricular arrhythmias (Lown grade 0-1). Group 2 was comprised of eight patients (26%) with significant ventricular arrhythmias (Lown grade 2-5). Twelve patients (39%) had supraventricular arrhythmias, three patients rare supraventricular tachycardia attacks, and seven patients occasional supraventricular ectopies. One patient had bradycardia-tachycardia attacks and one patient had junctional tachycardia. There was no correlation between age at the time of surgery and ventricular arrhythmias. Of the patients who had ventricular and supraventricular arrhythmias of various degrees on ambulatory monitoring, two had significant arrhythmias on routine electrocardiogram. Symptoms were rare in these patients. In conclusion, both supraventricular and ventricular arrhythmias were found in considerable frequency in our patients. As ventricular arrhythmias may be the cause of sudden death and supraventricular arrhythmias are a main cause of morbidity, it is important to evaluate ventricular and supraventricular arrhythmias by ambulatory monitoring in patients who have undergone correction of tetralogy of Fallot.

Subpulmonary and subaortic ridges in doubly committed subarterial ventricular septal defect: an echocardiographic study.

BACKGROUND: Certain conditions, including discrete subaortic stenosis and right ventricular outflow tract obstruction are well-known associations with ventricular septal defect. However, the association of discrete type subpulmonary and subaortic stenosis with ventricular septal defect has not been described to date. PATIENTS: In this report we present our experience in nine patients with doubly committed subarterial ventricular septal defect in whom associated discrete subpulmonary and subaortic stenosis were diagnosed by two-dimensional echocardiography. The mean age of the patients was 5.7 years, and eight of them were male. Two patients had additional hypertrophied anomalous muscle bundles with pressure gradients across the right ventricular outflow tract. Aortic valve prolapse was detected in two patients with one of them having mild aortic regurgitation. INTERVENTION: Cardiac catheterization was performed in four patients, and three underwent surgery. Doubly committed subarterial ventricular septal defect was closed, and subpulmonary and subaortic ridges were resected in each patient. Histological findings were similar for both ridges, which were composed of thin, short and irregularly arranged elastic fibres covered by endothelial cells. RESULTS: The finding of fibrous ridge on both sides of the doubly committed ventricular septal defect, where the turbulence is likely to be maximum, and the histopathological similarities of subpulmonary and subaortic ridges, may suggest a common mechanism for the development of fibrous ridges. CONCLUSION: Serial echocardiographic assessment of patients with doubly committed ventricular septal defect is recommended.

Pulmonary vascular sling responsible for esophageal and tracheal obstruction. A case report.

The disease of pulmonary vascular sling is a rare congenital condition. When it does occur it is often in conjunction with tracheal compression. The authors report herein a newborn with this anomaly presenting with a tracheal and esophageal compression.

Abnormal connection of the inferior vena cava to the left atrium with double outlet right ventricle and heterotaxia: a case report.

A 4-year-old boy with abnormal connection of the inferior vena cava to the left atrium and double outlet right ventricle and right atrial isomerism is presented. The anomalies were detected by echocardiography and angiography, and later verified through surgical intervention.

Coarctation of the aorta in infants and neonates: results and assessment of prognostic variables.

From 1984 until 1994, 75 consecutive patients younger than 12 months of age were operated on for coarctation of the aorta. We retrospectively analyzed predictive factors for morbidity and mortality, and also interaction between surgical procedures and recoarctation. Surgical procedures were as follows: resection with a traditional end-to-end (E-E) anastomosis in 55 patients (73.3%), prosthetic patch aortoplasty (PPA) in 12 patients (16%) and subclavian flap aortoplasty (SFA) in 8 patients (10.7%). Early mortality was 9.33% (7 patients). Logistic regression analysis proved that age at operation, associated anomalies of heart, type of coarctation, aortic arch hypoplasia and pulmonary banding were independent predictors of hospital death. Late mortality occurred in 7 patients (10.3%). Associated anomalies of heart were an independent prognostic factor for late mortality. Actuarial freedom from recoarctation at 1 year was 91% [confidence limits (CL): 82% to 97%] and 5 years were 74% (CL: 67% to 86%). Immediate postrepair gradient was equal after E-E anastomosis and other procedures. We conclude that the treatment of first choice in the management of coarctation of the aorta in infants is E-E anastomosis.

Congenital double-orifice mitral valve. A case report.

A three-year-old girl was admitted to Hacettepe Children's Hospital due to sweating and poor feeding. Echocardiography demonstrated ostium primum atrial septal defect with a cleft mitral valve. After two months of medical follow-up with digitalis, she was subjected to open-heart surgery, and a primum-type atrial septal defect with double mitral orifice was found. To avoid creating acute mitral stenosis, the cleft was only partially sutured and the septal defect was closed with a patch. Her postoperative recovery was uneventful. It is emphasized that surgical intervention should be individualized in each case.

Total correction for tetralogy of Fallot after Brock-type operation.

We present a case of tetralogy of Fallot who had previously undergone a graded Brock-type operation. The post-operative development of pulmonary arteries and pulmonary trunk after the Brock operation were very suitable for total correction, which was then performed successfully. In contrast to shunt, in Brock-type operations enlargement of the outflow tract induces symmetrical growth of hypoplastic pulmonary arteries without the risk of acquired pulmonary obstruction or peripheral stenosis at the site of anastomosis. After palliative operations, the repair can be deferred until the pulmonary arteries are of a suitable size.

Congenital absence of the pulmonary valve associated with pulmonary stenosis, large ductus arteriosus and intact ventricular septum. Case report.

A six-day-old neonate was diagnosed with a severe form of the syndrome of absent pulmonary valve associated with pulmonary stenosis, aneurysmal dilatation of the pulmonary artery and rare findings including an intact ventricular septum and large ductus arteriosus. The patient underwent surgical repair by closed technique. Cardiac catheterization data, hemodynamic and clinical findings, and surgical technique are reported. Congenital absence of the pulmonary valve is a rare cardiac anomaly. An especially severe form occurs with ventricular septal defect and pulmonary stenosis. The usual findings are respiratory distress, aneurysmal dilatation of the pulmonary arteries and pulmonary stenosis.

Surgical treatment in tetralogy of Fallot diagnosed by echocardiography.

The purpose of this paper is to present the authors' 3-yr experience of echocardiographic examination of patients with the clinical diagnosis of tetralogy of Fallot, and their evaluation for surgical treatment without prior cardiac catheterization. Among the patients with the clinical diagnosis of tetralogy of Fallot 227 had a definite diagnosis made by M-mode, two-dimensional, Doppler and contrast echocardiography. For the diagnosis of tetralogy of Fallot, ventricular septal defect, pulmonary stenosis, and overriding of the aorta were considered to be fundamental. Ventricular septal defect could be seen easily in the subaortic region by two-dimensional echocardiography. However, in some patients whose ventricular septal defect was not seen clearly, peripheral vein contrast echocardiography was performed. The diameters of pulmonary artery, and main branches at a few millimeters distal to their origin were measured. These parameters were correlated with the aortic diameter for evaluation as to whether they were able to accept the total cardiac output. In patients whose left ventricular end-diastolic dimension was small, shunt operation was preferred. In 115 patients the pediatric cardiologist performing the echocardiography thought that cardiac catheterization was necessary. In these cases the reliability of echocardiography in detecting important cardiac abnormalities was evaluated. Detection of ventricular septal defect, presence of pulmonary valve, detection of stenosis on the pulmonary bifurcation and/or main branches revealed a high sensitivity. Two-hundred-and-one patients diagnosed by echocardiography underwent total correction. In all cases except one the preoperative diagnosis was confirmed by surgery.(ABSTRACT TRUNCATED AT 250 WORDS)

Two-dimensional and Doppler echocardiography in the diagnosis of absent pulmonary valve syndrome (surgery without catheterization).

The majority of severely affected infants with absent pulmonary valve syndrome develop varying degrees of respiratory distress. Cardiac catheterization and angiocardiography are still performed in the diagnosis of these infants prior to surgery. However, considering the high risks of these invasive investigations for severely symptomatic infants, diagnosis using only noninvasive methods becomes important. In this regard, we present three cases of absent pulmonary valve syndrome, diagnosed pre-operatively by both two-dimensional and Doppler echocardiography. The diagnosis was confirmed by cardiac catheterization and angiocardiography in two cases and by surgery in a symptomatic infant. Therefore, we are of the opinion that the two-dimensional and Doppler echocardiographic methods are most reliable in diagnosing absent pulmonary valve syndrome, and that severely symptomatic infants can be referred for surgery without catheterization.

Two-dimensional and Doppler echocardiographic findings of congenital coronary artery fistula.

Three patients with coronary artery fistula confirmed by coronary angiography and surgery were studied using two-dimensional and Doppler echocardiography. Two of these patients had left coronary artery-right ventricle fistula. The proximal dilatation of the coronary arteries was visualized in all three patients by two-dimensional echocardiography. The course of the dilated right coronary artery and left circumflex artery was demonstrated by two-dimensional echocardiography in two patients. We were able to detect the disturbed flow at the drainage site in one patient by using the conventional Doppler echocardiography. In conclusion, it should be emphasized that two-dimensional and Doppler echocardiography can be useful methods in diagnosing coronary artery fistula.

Congenital absence of pulmonary valve leaflets with intact ventricular septum in a neonate.

Symptomatic infants with congenital absence of pulmonary valve leaflets suffer primarily from respiratory insufficiency caused by bronchial compression by the dilated pulmonary arteries, and have a high mortality rate. We report the successful treatment of absent pulmonary valve syndrome with intact ventricular septum in a neonate. The treatment consisted of resection of the pulmonary artery aneurysm and enlargement of the pulmonary annulus.

Peripheral vein contrast echocardiography in atrial septal defect.

This study was carried out on 233 children suspected clinically of having atrial septal defect with the aim of investigating the diagnostic capability of peripheral venous contrast echocardiography. The transfer of contrast material from the right atrium into the left atrium was evaluated as "positive contrast", while noncontrast blood, passing from the left atrium into the right atrium was termed "negative contrast". Positive contrasts were quantitated in four grades. A significant negative contrast effect was graded 3- or 4-. Three positive, 4+ and/or 3-, 4- contrast effects were considered definite evidence of an atrial septal defect. Among the cases with the above findings 92 underwent surgical closure of atrial septal defect. The procedure was successful in all patients operated; the size of the defect was large. This result demonstrates that the method applied is a safe and reliable one. However, in a group of cases without the above echocardiographic findings the presence of an atrial septal defect was detected by cardiac catheterization and angiocardiography. Therefore, we can conclude that the method applied is not a sensitive, but a specific one, for definite detection of atrial septal defect.