Percutaneous transhepatic stenting of a restrictive atrial septal communication in hypoplastic left heart syndrome

The postnatal survival of patients with congenital cardiac defects such as hypoplastic left heart syndrome (HLHS) is dependent on the patency of the ductus arteriosus and the presence of an unrestrictive atrial septal defect (ASD). We report a six week old infant with HLHS and tricuspid valve regurgitation with restrictive ASD. Transfemoral balloon atrial septostomy provided temporary relief but further attempts were not possible. A transhepatic venous approach to stent the atrial communication was technically successful. This approach may be the only access for certain infant cardiac catheterization interventions, permitting the use of a large delivery system (≥ 8 Fr). © 2014 Wiley Periodicals, Inc.     

Intrauterine pulmonary venous flow and restrictive foramen ovale in fetal hypoplastic left heart syndrome

OBJECTIVES: We sought to determine whether direct foramen ovale (FO) assessment or pulmonary venous (PV) flow patterns in fetal hypoplastic left heart syndrome (HLHS) correlate with clinical markers of postnatal left atrial (LA) hypertension severity associated with restrictive FO. BACKGROUND: Restrictive FO places a newborn with HLHS at high risk of mortality and morbidity. METHODS: We reviewed the prenatal and postnatal echocardiograms and outcomes of 45 fetuses with variants of HLHS diagnosed since May 1999 to determine whether direct FO assessment or PV flow patterns correlate with clinical LA hypertension after birth. RESULTS: Direct FO assessment in utero showed a poor correlation with postnatal FO size, Pao(2), base excess, and the need for atrial septoplasty (p > 0.05). In 40 fetuses with available PV spectra, three PV flow patterns were observed: 1). continuous forward flow with a small a-wave reversal (velocity time integral [VTI] for reverse/forward flow [VTIR/VTIF ratio <0.18]); 2). continuous forward flow with increased a-wave reversal (VTIR/VTIF ratio >or=0.18); and 3). brief to-and-fro flow. Among 19 live-borns, the postnatal FO diameter was smaller in patients with type B than in those with type A flow (1.6 +/- 1.6 mm and 4.5 +/- 2.1 mm, respectively; p = 0.0015), and all patients with type C flow had an intact atrial septum. All three patients with type C flow were critically ill at birth, requiring emergent atrial septoplasty, and two died after heart transplantation, whereas patients with type A or B flow were clinically stable, with only one postoperative death. CONCLUSIONS: Prenatal PV flow patterns in HLHS identify the fetus at risk of severe LA hypertension at birth.     

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.     

Atrial septal interventions in patients with hypoplastic left heart syndrome

Objectives: To report an institutional experience performing percutaneous atrial septal interventions in patients with hypoplastic left heart syndrome (HLHS). Background: The success of the Hybrid approach in palliating patients with HLHS is crucially dependant on relieving any significant interatrial restriction. Data on transcatheter interventions to relieve atrial septal restrictions in patients with HLHS are limited. Methods: We retrospectively reviewed 67 transcatheter atrial septal interventions that were performed between July 2002 and September 2007 in 56 patients with HLHS. The median weight was 3.35 kg. About 10.7% of patients had an intact atrial septum. Balloon atrial septostomy (BAS) was used in 77.6% of procedures, additional techniques in 35.8% of procedures. Patients were divided into those with standard atrial septal anatomy (group A, n = 33) and those with complex atrial septal anatomy (group B, n = 23). Results: The mean trans‐septal gradient was reduced significantly from 7 mm Hg to 1 mm Hg with the median time to discharge being 3.5 days. Major adverse events were seen in 8.9% of procedures, whereas minor adverse events occurred in 26.8% of procedures. Adverse events were significantly less common in patients with standard atrial septal anatomy, compared to those with complex atrial septal anatomy (25.6% versus 50.0%). About 19.6% patients required repeated atrial septal interventions. Survival up to and including Comprehensive stage II palliation was 73% group A, and 57% in group B. Conclusions: With utilization of appropriate techniques and equipment, atrial septal interventions in HLHS can be performed successfully in virtually all patients. Complex atrial septal anatomy is technically challenging and has a higher incidence of procedural adverse events. In the majority of patients, standard BAS can be performed safely, and is usually the only intervention required to achieve adequate relief of atrial septal restriction until Comprehensive stage II palliation. © 2008 Wiley‐Liss, Inc.     

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.     

Stenting of a restrictive foramen ovale in a patient with hypoplastic left heart syndrome

Neonates with a severely restrictive foramen ovale and hypoplastic left heart syndrome (HLHS) present as an emergency. We report the case of a newborn in whom left atrial decompression was successfully achieved by stent implantation into the foramen ovale on the first day of life.     

Catheter-based decompression of the left atrium in patients with hypoplastic left heart syndrome and restrictive atrial septum is safe and effective.

Infants with hypoplastic left heart syndrome (HLHS) and restrictive or intact atrial septum (rAS) present with cyanosis, pulmonary edema, and are critically ill. A previous report from our institution on emergent Norwood for HLHS with rAS showed 10% survival. We hypothesized that transcatheter left atrial (LA) decompression in HLHS with rAS would safely and effectively relieve LA hypertension, improve oxygenation, and improve Norwood survival. Between 1996 and 2004, 30 patients with HLHS and rAS underwent cardiac catheterization for pre-Norwood intervention. Twenty-eight atrial septostomies were performed: 23 static balloon dilations, 4 Rashkind septostomies, and 1 intra-atrial stent. Two procedures were aborted due to perforation (n = 1) or inability to enter the LA (n = 1). Eight total patients required surgical septectomy, for a failure rate of 27%. There were no catheter-related mortalities, although two patients died within 36 hr of the procedure after surgical septectomy. Major complications occurred in three patients (10%)--atrial perforations requiring intervention. Mean atrial septal defect gradient fell from 16.7 +/- 4.9 to 6.3 +/- 3.4 mm Hg (P < 0.001; n = 18). Mean LA pressure dropped from 21.8 +/- 5.5 to 13.1 +/- 6.5 mm Hg (P < 0.001; n = 16). Mean PaO(2) rose from 29.5 +/- 9.1 to 36.5 +/- 5.1 torr (P < 0.001; n = 23). Seventeen of 30 patients (57%) survived to discharge from Norwood. Thirteen have undergone hemi-Fontan and nine Fontan. Sixteen of 22 successful decompressions (73%) survived to discharge. Transcatheter decompression of the LA for patients with HLHS and rAS can be performed safely, reduces the transatrial gradient, and improves oxygenation. Catheter intervention improves survival compared to historical controls undergoing emergent Norwood.     

Pattern of pulmonary venous blood flow in the hypoplastic left heart syndrome in the fetus

OBJECTIVE: To determine whether restriction at the atrial septum in the newborn with hypoplastic left heart syndrome can be predicted accurately by examining the pattern of pulmonary venous flow in the fetus. A restrictive atrial septum can contribute to haemodynamic instability before surgery for this lesion and has been associated with an increased mortality. DESIGN: Pulmonary venous pulsed Doppler tracings were compared between fetuses with hypoplastic left heart syndrome and controls. The size of the atrial septal defect on the postnatal echocardiogram was graded according to the degree of restriction. Pulsed Doppler tracings of pulmonary venous blood flow were obtained in 18 fetuses with left atrial outflow atresia and compared with 77 controls, adjusted for gestational age. Postnatal echocardiograms were available for analysis in 13 of 18 neonates. SETTING: A tertiary referral centre for fetal cardiology and paediatric cardiac surgery. RESULTS: Fetuses with hypoplastic left heart syndrome were different from controls in all pulmonary vein indices measured. As assessed from the postnatal echocardiogram, there were seven fetuses with a restrictive atrial septum. In these fetuses, the systolic flow velocity (p < 0.01), S/D ratio (p < 0.01), and peak reversal wave (p < 0.001) in the pulmonary vein tracing showed a good correlation with the degree of restriction. CONCLUSIONS: The Doppler pattern of pulmonary venous flow in the fetus with hypoplastic left heart syndrome appears to be a reliable predictor of restriction of the atrial septum in the neonate. This may help in the immediate post-delivery management of these infants before surgery.     

成人房间隔缺损合并肺动脉高压的缺损闭合治疗

随着成人先天性心脏病临床处理指南的更新,成人房间隔缺损合(ASD)合并肺动脉高压(P H)的缺损闭合治疗策略有了新进展.该文简述成人ASD合并P H的临床分类及特点,重点介绍ASD合并P H的缺损闭合策略,包括常规缺损闭合、"药物到缺损闭合"策略以及带孔封堵器封堵术等.     

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.     

Differential pulmonary flow in hypoplastic left heart syndrome.

We report a case of hypoplastic left heart syndrome associated with restrictive interatrial communication and partial anomalous pulmonary venous connection via a right lower pulmonary vein draining to the inferior vena cava. We found unequal pulmonary artery pressure and different pulmonary artery structure, with the right pulmonary artery being lower in pressure and more tortuous and dilated in its peripheral branches than the left. This was attributed to the variant degrees of pulmonary venous obstruction. The left pulmonary venous return was severely obstructed by the restrictive interatrial communication, whereas the anomalous right lower pulmonary vein drained into the inferior vena cava, with less obstruction. To the best of our knowledge, there has never before been a report of differential pulmonary flow associated with a partial anomalous pulmonary vein, in a case of hypoplastic left heart syndrome.