Right-sided endocarditis complicating an atrial septal defect

We report the case of a 45-year-old male patient not addicted to narcotic who presented with an infective endocarditis on the pulmonary valve. He was found to have an uncomplicated secundum-type atrial septal defect. This association is an extremely rare event.     

Natural histories of atrial septal defect with pulmonary hypertension, and ventricular septal defect with pulmonary hypertension

A study of the natural history of 51 adult patients with atrial septal defect with pulmonary hypertension (ASD + PH) was performed. ASD + PH of less than 14 Um2 of pulmonary artery resistance (PVR) was considered an indication for surgery. The prognosis of surgically treated patients was favorable, but that of medically treated patients was poor. For patients with ventricular septal defect with pulmonary hypertension (VSD + PH), surgery was considered for pulmonary-systemic vascular resistance ratio (Rp/Rs) less than 0.5, and for patients under than 10 years and, ideally, under 2 years of age.     

Frequency of severe pulmonary hypertension complicating "isolated" atrial septal defect in infancy

Atrial septal defects (ASDs) are typically asymptomatic in infancy and early childhood, and elective defect closure is usually performed at ages of 4 to 6 years. Severe pulmonary hypertension (PH) complicating an ASD is seen in adulthood and has only occasionally been reported in small children. A retrospective study was undertaken to evaluate the incidence of severe PH complicating an isolated ASD and requiring early surgical correction. During a 10-year period (1996 to 2006), 355 pediatric patients underwent treatment for isolated ASDs either surgically or by catheter intervention at 2 tertiary referral centers. Two hundred ninety-seven patients had secundum ASDs, and 58 had primum ASDs with mild to moderate mitral regurgitation. Eight infants were found with isolated ASDs (6 with secundum ASDs and 2 with primum ASDs) associated with significant PH, accounting for 2.2% of all patients with ASDs at the centers. These 8 infants had invasively measured pulmonary artery pressures of 50% to 100% of systemic pressure. They were operated in the first year of life and had complicated postoperative courses requiring specific treatment for PH for up to 16 weeks postoperatively. The ultimate outcomes in all 8 infants were good, with persistent normalization of pulmonary pressures during midterm follow-up of up to 60 months (median 28). All other patients with ASDs had normal pulmonary pressures, and the mean age at defect closure was significantly older, at 6.2 years for secundum ASDs and 3.2 years for primum ASDs. In conclusion, ASDs were rarely associated with significant PH in infancy but then required early surgery and were associated with excellent midterm outcomes in these patients.     

Atrial septal defect with right to left shunt despite normal pulmonary artery pressure

A 74 year old woman had right to left shunting through an atrial septal defect despite normal right heart pressures. Acute volume expansion temporarily reduced the shunt. Contrast echocardiography and angiography demonstrated that this shunting occurred almost exclusively from the inferior vena cava. At surgery a redundant flap of septum secundum was found that was adjacent to the inferior vena cava orifice, intercepting its blood return like a spinnaker and shunting it into the left atrium.     

Preferential shunting of venous return from normally connected left pulmonary veins in secundum atrial septal defect

Preferential shunting of left pulmonary venous blood across a secundum atrial septal defect, a paradoxical hemodynamic finding, was documented in six patients by indicator-dilution techniques. Subsequent surgical findings excluded anomalous connection of the left pulmonary veins, which can result in similar or identical dye curve patterns. An adequate anatomic explanation for this physiologic paradox was apparent In only one patient.     

Successful repair of an atrial septal defect associated with right to left shunting

An atrial septal defect was successfully repaired in a young woman despite the presence of pulmonary hypertension and right to left shunting. Before repair both isoprenaline infusion and 100% inspired oxygen produced significant falls in pulmonary artery pressure and pulmonary vascular resistance. A lung biopsy specimen at operation indicated a considerable decrease in the concentration of parenchymal pulmonary arteries and an absence of intimal fibrosis or medial hypertrophy. Pulmonary artery banding performed in infancy, as part of the management of a ventricular septal defect, may have contributed to the underdevelopment of the pulmonary vascular tree. The reduced number of pulmonary arteries is a possible explanation for the pulmonary hypertension.     

Three overlapping septal occlusion devices to treat residual shunting across an atrial septal defect

A 46-year-old woman with a history of non-ischaemic cardiomyopathy,severe biventricular failure treated with implantable cardiacdefibrillator, presented with increasing dyspnoea. She alsohas a history of diffuse scleroderma and     

Successful repair of an atrial septal defect associated with right to left shunting.

An atrial septal defect was successfully repaired in a young woman despite the presence of pulmonary hypertension and right to left shunting. Before repair both isoprenaline infusion and 100% inspired oxygen produced significant falls in pulmonary artery pressure and pulmonary vascular resistance. A lung biopsy specimen at operation indicated a considerable decrease in the concentration of parenchymal pulmonary arteries and an absence of intimal fibrosis or medial hypertrophy. Pulmonary artery banding performed in infancy, as part of the management of a ventricular septal defect, may have contributed to the underdevelopment of the pulmonary vascular tree. The reduced number of pulmonary arteries is a possible explanation for the pulmonary hypertension.     

Right-to-left interatrial shunt despite normal pulmonary artery pressure. Anatomical implications

Platypnea-orthodeoxia is a rare syndrome characterized by dyspnea induced by the upright position and relieved by supine position and an arterial deoxygenation increased by the upright position which improves during recumbency. In many cases, this syndrome has been associated with patent foramen ovale and right-to-left shunt. Several anatomical factors that can alter the atrial anatomy and facilitate shunting through an interatrial communication have been related with this syndrome. We present a case in which an enlarged aortic root was the main anatomical factor that contributed to transient right-to-left shunting induced by postural changes.     

An echocardiographic study of perimembranous ventricular septal defect with left ventricular to right atrial shunting

Twenty nine patients with isolated perimembranous ventricular septal defects were investigated by M mode, cross sectional, and pulsed Doppler echocardiography. Tricuspid valve anomalies were present in all six patients with a left ventricular-right atrial shunt but in only six (26%) of 23 patients who had interventricular shunts only. Systolic flutter of the tricuspid valve was shown in five (83%) of the six patients with a ventriculoatrial shunt but not in the other patients. Systolic turbulence in both the right ventricle and right atrium was detected by Doppler echocardiography only in patients with ventriculoatrial shunting. A perimembranous ventricular septal defect with left ventricular to right atrial shunt can be diagnosed by its combined M mode, cross sectional, and pulsed Doppler echocardiographic features.     

Pulmonary vascular disease in secundum atrial septal defect with pulmonary hypertension

Histopathologic and morphometric studies of small pulmonary arteries were performed in 16 cases of secundum atrial septal defect (ASD) and severe pulmonary hypertension. Besides typical plexogenic arteriopathies found in six cases, organized microthrombi and what we call "musculoelastosis," that is, proliferation of longitudinal smooth muscle bundles and elastic fibers, were seen in small pulmonary arteries in three and four cases, respectively. These changes were observed co-existing in the remaining three cases. Thrombi of the small pulmonary arteries and musculoelastosis were the forms of pulmonary vascular disease characteristically found in the older patients with both ASD and pulmonary hypertension. Yamaki's index of pulmonary vascular disease, though effective in describing severity of plexogenic arteriopathy, proved to be less so for the intimal lesions in old microthrombi and for musculoelastosis. Among the cases with plexogenic arteriopathy, there was a positive significant correlation between the medial thickness of small pulmonary arteries and peak arterial pressure, which, however, was not demonstrated when all the cases of ASD were included.     

Interatrial shunting in atrial septal aneurysm

Adequate contrast 2-dimensional (2-D) echocardiograms were recorded in 13 to 16 patients with typical 2-D findings of atrial septal aneurysm. Five patients were referred for detection of intracardiac source of emboli after embolic stroke and 11 were evaluated for suspicion of valvular or other forms of heart disease. Contrary to findings of previous clinical studies, all 13 patients had 2-D evidence of right-to-left atrial level shunting. These findings represent the first clinical evidence of a high prevalence of atrial shunting in patients with atrial septal aneurysm.     

Persistent iatrogenic atrial septal defect after pulmonary vein isolation

INTRODUCTION: Pulmonary vein isolation (PVI) is widely practiced for treating atrial fibrillation. Transseptal access is necessary with one or even more transseptal sheaths to perform PVI. METHODS: In this prospective study, 31 patients were examined with transesophageal echocardiography before, immediately after and in a 3-, 6- and 12-month follow-up period for evaluation of iatrogenic atrial septal defect (iASD). All patients underwent PVI with double transseptal puncture. RESULTS: An iASD was detected in 27/31 patients (87%) with a maximum diameter of 1.0 mm. After 3 months, the iASDs were completely closed in 26/27 (96.3%) patients. In 1/27 (3.7%) patients, there was an iASD detectable even after 12 months. Clinically no patient suffered from cerebral or cardiac embolism in the follow-up period. CONCLUSIONS: We were only able to find small iASDs with left to right shunting after PVI but none with right to left shunting. iASDs following PVI show a high spontaneous closure rate already at 3 months of follow-up and are not associated with an increased rate of paradoxical embolism.     

Concurrent percutaneous atrial septal defect closure and pulmonary valvuloplasty

Severe pulmonary valve stenosis in association with a large atrial septal defect is uncommon. Simultaneous percutaneous closure of the atrial septal defect and pulmonary valvuloplasty appears to be an attractive modality when compared to patients with these two lesions being subjected to surgery or being treated percutaneously at different sittings. We encountered two cases, both with significant pulmonary valvular stenosis associated with atrial septal defect (fossa ovalis type). Both lesions were successfully treated percutaneously in the same sitting. Pulmonary valvuloplasty was done initially and the atrial septal defect closed later to minimize catheter manipulation after device implantation.