The second heart sound in atrial septal defect

Ventricular and arterial pressure tracings, electrocardiograms and phonocardiograms were studied in 4 normal subjects and 52 patients with atrial septal defect. After excluding cases in which complicating factors were involved, 10 cases of secundum type and 3 cases of primum type were studied in detail.     

A case of successful surgery of atrial septal defect combined with coronary artery bypass grafting

A 66-year-old man, who had atrial septal defect (ASD) associated with severe coronary artery disease, underwent closure of ASD and concomitant coronary artery bypass grafting (LIMA-LAD & SVG-RCA). Response to surgery was excellent, with improvement of cardiac function and returning to NYHA functional class I, while, preoperatively, the patient had been limited due to symptoms related both to large left to right shunt flows (Qp/Qs 3.04) and to left ventricular dysfunction because of severe myocardial ischemia as well as previous inferior myocardial infarction. SAD is the most common form of congenital heart disease requiring surgery in adults, accordingly it should be remembered that some of the elderly patients with ASD might have obvious or occult coronary artery disease at the time of diagnosis and operative intervention.     

Normal splitting of the second heart sound in atrial septal defect

A case is presented of a patient with proved ostium secundum atrial septal defect and a large left to right shunt without associated anomalous pulmonary venous return or pulmonary hypertension who demonstrated a second heart sound that was widely split with inspiration and became single with expiration.

Evidence supporting the constancy of a wide fixed split of S₂ with atrial septal defects is presented; the importance of the specific type of interatrial septal defect and the presence or absence of associated pulmonary hypertension is emphasized.

The various theories proposed to explain the wide fixed splitting of S₂ with atrial septal defect are reviewed and discussed with particular reference to the case presented.     

介入治疗房间隔缺损并发肺动脉瓣狭窄的临床评价

目的:评价介入治疗房间隔缺损并发肺动脉瓣狭窄的近中期疗效。方法:11例患者,年龄3～53（16±15）岁。超声心动图测量房间隔缺损最大直径为8～38（21.7±10.8）mm,1例心房水平显示双向分流,10例呈左向右分流,均并发有不同程度的肺动脉瓣狭窄。先行球囊肺动脉瓣成形术（PBPV）,其中6例采用肺动脉瓣单球囊,5例采用Inoue二尖瓣球囊扩张;而后置入Amplatzer型房间隔缺损封堵器闭合缺损,选择的封堵器直径为10～40（22.9±11.5）mm。结果:同期行介入治疗均成功,PBPV术后即刻右室收缩压由术前77±18mmHg降至39±7mmHg,肺动脉至右心室跨瓣压差由术前47±18mmHg降至9±5mmHg（P<0.01）。术后超声心动图随访3～67个月,疗效满意,房间隔未见残余分流。结论:介入方法治疗房间隔缺损并肺动脉瓣狭窄安全、有效。     

Familial recurrence of congenital heart disease in patients with ostium secundum atrial septal defect.

AIMS: Ostium secundum atrial septal defect (osASD) is one of the most common cardiac malformations. Few data are available on the familial recurrence of congenital heart disease (CHD), in particular, in a large group of patients with isolated osASD. The aim is to investigate the familial recurrence of CHD in up to third-degree relatives from a large sample of consecutively enrolled patients with osASD, taking into account the influence of degree of relatedness (as number of relatives). METHODS AND RESULTS: From January 1998 to December 2002, we enrolled 583 patients with osASD and 408 healthy subjects, referred to our tertiary centre. We hypothesized that a positive family history required at least one relative with CHD to constitute a risk factor. In this model of analysis, the null hypothesis is a similar familial history between cases and controls. Among 583 patients with osASD, 109 (19%) had at least one relative with CHD. Among the 408 healthy subjects studied, only 23 (6%) had a family history of CHD. A familial recurrence of CHD was demonstrated in 72 of 312 (23%) patients with isolated osASD and in 37 of 271 (13.6%) patients with non-isolated osASD. Familial recurrence of isolated osASD was demonstrated in 22 of 312 patients (7%) with an isolated osASD and only in six of 271 patients (2.2%) with non-isolated osASD. The familial recurrence risk of isolated osASD in patients with isolated osASD was higher in sibs, especially in sisters (33.3%). CONCLUSION: This study underscores the role of genetic factors in the determination of CHD, particularly osASD. Our results could represent the basis for further studies to calculate a 'value of family history' to adapt the familial recurrence to the real size of each family group. In this way, we could select families with a 'tendency' to develop CHD, particularly osASD. In these families, we could analyse the genetic pattern to establish abnormalities and the bases of CHD.     

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.     

Syndrome of valvular pulmonary stenosis and valvular aortic stenosis with atrial septal defect

Clinical and morphological findings are described in 2 adults with combined valvular pulmonary and aortic stenosis with atrial septal defect, an extremely rare combination of lesions. The direction of the shunt via the atrial septal defect in this combination appears to be dependent on the relative degree of outflow obstruction on the right side compared with the left side of the heart. In 1 patient the degree of obstruction at the pulmonary valve was nearly three times that at the aortic valve and, therefore, the shunt was right to left and the patient was severely cyanotic. In the other patient, the degree of obstruction at the pulmonary valve was slightly less than that at the aortic valve and, therefore, the shunt was entirely left to right and the patient was acyanotic. Diagnosis of an associated obstructive lesion on the left side of the heart in a patient with an obstructive lesion on the right side of the heart is essential because operative relief of the right-sided obstruction without relief of the left-sided obstruction may lead to rapid, fatal pulmonary congestion.     

Syndrome of valvular pulmonary stenosis and valvular aortic stenosis with atrial septal defect.

Clinical and morphological findings are described in 2 adults with combined valvular pulmonary and aortic stenosis with atrial septal defect, an extremely rare combination of lesions. The direction of the shunt via the atrial septal defect in this combination appears to be dependent on the relative degree of outflow obstruction on the right side compared with the left side of the heart. In 1 patient the degree of obstruction at the pulmonary valve was nearly three times that at the aortic valve and, therefore, the shunt was right to left and the patient was severely cyanotic. In the other patient, the degree of obstruction at the pulmonary valve was slightly less than that at the aortic valve and, therefore, the shunt was entirely left to right and the patient was acyanotic. Diagnosis of an associated obstructive lesion on the left side of the heart in a patient with an obstructive lesion on the right side of the heart is essential because operative relief of the right-sided obstruction without relief of the left-sided obstruction may lead to rapid, fatal pulmonary congestion.     

Multiperforated atrial septal aneurysm and a single coronary artery

Multiperforated atrial septal aneurysm (ASA) with left-to-right shunt and elevated pulmonary arterial pressure is rare. We present an adult patient with this unusual anomaly combined with a single coronary ostium emerging from the right sinus of Valsalva. The ASA was excised and the defect repaired successfully.     

Percutaneous closure of secundum atrial septal defect in adults a single center experience with the amplatzer septal occluder

Percutaneous closure of atrial septal defects (ASD) is increasingly performed in pediatric and adult patients. This study reports a single-center experience with the use of the Amplatzer Septal Occluder for percutaneous ASD closure in adults. One hundred and seventeen consecutive patients were prospectively included in the database. The procedure was successful in 106 patients (90.6%). The mean age was 50 years. Eighty-four percent of the patients had a Qp:Qs > 1.5. Other indications included a history of thromboembolism, decreased exercise tolerance, atrial arrhythmias, evidence of pulmonary hypertension or right heart overload. Mean procedure time was 44 minutes, and mean fluoroscopy time was 13 minutes. The mean defect diameter was 17.2 mm (range, 5 30 mm). There was no residual shunting at the end of the procedure in 75% of the patients. Shunting was minimal in all but 2 of the remaining 25 patients (1.8%). No major complications were observed at a mean follow-up of 19 months. Patients in whom the procedure failed had larger defects (mean, 25.0 mm versus 18.3 mm; p < 0.001), larger right ventricles (44.1 mm versus 38.2 mm; p = 0.03) and more significant shunting (Qp:Qs, 3.5 versus 2.1; p < 0.001). New York Heart Association functional class improved in all but 1 patient. Percutaneous ASD closure using ASO is safe and mid-term results compare favorably with those reported with surgical ASD closure. It can therefore be recommended as the first line of treatment in adult patients with secundum ASD.     

杂交手术治疗冠心病合并房间隔缺损二例报告

1临床资料 患者男性,67岁,因“体检时发现心脏杂音”入院。患者无特殊不适主诉。入院查体：Bp130／80mmHg（1mmHg=0．133kPa）,心率70次／min,偶发早搏,双肺呼吸音粗,未及干、湿哕音,胸骨左缘Ⅱ肋间可闻及Ⅲ／6级收缩期杂音,腹软,无压痛,肝脾未及,双下肢不肿。辅助检查：肌酸激酶同工酶（CK—MB）12U／L,心肌肌钙蛋白Ⅰ（cTnI）0．01ng／ml。心电图：不完全右束支传导阻滞,偶发房性早搏。经胸超声心动：房间隔中部缺失30min,左室舒张末径38mm,EF值60％,FS24％。心功能Ⅱ级（Killip分级）。     

Acute left ventricular failure after transcatheter closure of a secundum atrial septal defect in a patient with coronary artery disease: a critical reappraisal.

We report a case of acute left ventricular failure after transcatheter closure of a single secundum atrial septal defect in a 68-year-old man with coronary artery disease. Just before the procedure, two coronary lesions had been treated with direct stenting. Transcatheter closure of atrial septal defects should always be deferred in ischemic heart disease patients who need percutaneous myocardial revascularization.     

经静脉闭合房间隔缺损的疗效观察

目的 :评价经静脉置入 Amplatzer封堵器治疗继发孔型房间隔缺损 (ASD)的疗效。方法 :2 4例患者术前经多普勒超声心动图检查 ASD直径为 6～ 30 (18.96± 7.0 3) m m。在透视及经超声心动图引导下经静脉置入Am platzer封堵器闭合 ASD。结果 :2 4例 ASD直径的球囊测量值为 15～ 36 (2 6 .2 5± 7.5 7) mm ,选择的封堵器直径为 14～ 36 (2 6 .5 2± 7.15 ) m m。 2 4例封堵器置入均获得成功 ,术中无并发症 ,1例在术后第 3天出现 度 型房室传导阻滞 ,2周后恢复。 7例术后即刻超声检查显示微量残余分流 ,术后 1周复查均无分流。结论 :经静脉置入 Amplatzer封堵器治疗 ASD是一种有效的非外科手术方法。     

Left main coronary artery and aortic root compression associated with atrial septal defect and pulmonary hypertension

The extrinsic compression of left main coronary artery (LMCA) by dilated pulmonary artery is rarely reported. Various congenital and acquired diseases were shown to cause extrinsic LMCA compression. Here we present a child with aortic root and LMCA compression due to dilated pulmonary trunk and causing angina like chest pain. The patient had also electrocardiographic evidence of myocardial ischemia. This case report will be a guide for the evaluation and surgical treatment of the patients with pulmonary hypertension and LMCA compression.     

A quadricuspid aortic valve with atrial septal defect

A quadricuspid aortic valve (QAV) is a rare congenital heart defect, previously described as an incidental finding at the time of surgery or postmortem, which is now being increasingly detected by 2D transthoracic or transesophageal echocardiogram. With advances in echocardiography, secondary cardiac anomalies are also being described in association with QAV. Herein we describe a patient with QAV with a secundum atrial septal defect.