Successful Treatment of an Adult Patient with an Aortopulmonary Window and Severe Unilateral Pulmonary Hypertension

A 40‐year‐old woman with an aortopulmonary window combined with a severe stenosis of the right pulmonary artery was successfully treated by surgical closure of the defect and pulmonary artery patch plasty of the pulmonary stenosis. Even though the vasculature of the left lung was severely damaged preoperatively, the resulting pressure in the lung after surgical correction was only mildly elevated.     

Root Dilation in Patients with Truncus Arteriosus

Objective. A dilated aortic root is a common finding in children and adults with some forms of congenital heart defects. No data exist on root dilation in truncus arteriosus. We sought to delineate root dimensions across a population of patients with truncus arteriosus. Design. We performed a single‐center retrospective review of all patients with truncus arteriosus. Demographic information, clinical history, and most recent echocardiographic data were evaluated. Results. We identified 76 patients whose most recent study was at a median age of 5.4 years (range 0–32.7 years). Mean truncal root z‐score was 5.1 ± 2.3. All but three patients had truncal root z‐scores greater than or equal to 2. Truncal root z‐scores remained stable with increasing body surface area and age. There were no cases of dissection or rupture. Six patients underwent truncal root surgery, typically for indications of root dilation with significant truncal valve insufficiency and left ventricular dilation. Conclusions. In conclusion, mean truncal root z‐score was 5, and all but three patients had truncal root z‐scores greater than or equal to 2. Although repeat surgical intervention was rare and major complications related to root dilation did not occur in our cohort, further studies with longitudinal follow‐up into adulthood are needed.     

An Unusual Cause of Hemoptysis—Aberrant Origin of the Left Pulmonary Artery from the Ascending Aorta in the Adult

Aberrant origin of a pulmonary artery from the ascending aorta is an uncommon congenital vascular malformation with poor survival without surgery. In this case report, we describe the unusual late diagnosis of this congenital malformation in an otherwise asymptomatic young man presenting with mild hemoptysis. We review the natural and modified history of this defect and the relevant aspects of follow-up in adult life.     

Clinical Features of the Complete Closure of the Ductus Arteriosus Prenatally

Objective. Prenatal constriction of the ductus arteriosus associated with maternal drug ingestion was reported several decades ago. There are fewer reports of the complete closure of the ductus arteriosus; therefore, the clinical features of the latter are poorly understood. The aim of this study is to clarify the clinical features of complete ductal closure and postnatal pulmonary hypertension by performing echocardiography of the fetus. Patients. We diagnosed four fetuses with complete ductal closure by performing fetal echocardiography and reviewed the prenatal and postnatal medical records of the mother and fetus. Results. One mother each had bronchial asthma, ulcerative colitis, and idiopathic thrombocytopenic purpura, and they had received nonsteroidal anti‐inflammatory drugs and/or corticosteroids during pregnancy. The fourth mother did not have basal disease and had not ingested any drugs. Fetal diagnosis was performed at 32–38 weeks of gestation. All fetuses had right heart dilatation with tricuspid regurgitation in the absence of any cardiac defects, and Doppler echocardiography indicated that the right ventricular pressure was elevated. Two of the fetuses had fetal hydrops, which suggested severe right heart dysfunction. All fetuses were delivered by emergent cesarean delivery. After birth, all the infants developed persistent pulmonary hypertension and required oxygen inhalation. Of these, three required mechanical ventilation, and two, nitric oxide inhalation. All infants improved within 2 weeks, and they had no neurological and cardiac complications after discharge. Conclusion. Right heart dilatation and severe tricuspid regurgitation in the absence of a cardiac defect in the fetus strongly suggested ductal dysfunction. Careful evaluation of ductal patency and right ventricular function can lead to precise early diagnosis and good prognosis.     

Congenital Right Pulmonary Artery Agenesis with Atrial Septal Defect and Pulmonary Hypertension

Unilateral pulmonary artery agenesis is a rare congenital anomaly caused by a backward displacement of the conical artery of the truncus arteriosus. It is commonly associated with additional cardiovascular abnormalities. A 7‐year‐old girl was admitted to our clinic with the complaint of shortness of breath upon exertion. Chest radiography revealed a hypoplastic right lung. Absence of the right pulmonary artery with atrial septal defect and pulmonary hypertension was demonstrated by echocardiography, computed tomography, and cardiac catheterization. Bosentan is effectively used to treat pulmonary arterial hypertension.     

Percutaneous Interventions in Adults with Complex Cyanotic Congenital Heart Disease

Background. Adult patients with complex cyanotic heart disease present unique anatomic and physiological challenges. Changes in pulmonary blood flow over time may cause progressive cyanosis and exercise intolerance. The utility of percutaneous intervention in palliating adults with complex anatomy is unknown. Methods. This is a single‐center case series of percutaneous interventions in adults with complex cyanotic heart disease. Six patients (5 males, 1 female) underwent a total of 9 percutaneous interventions. Three patients required a second intervention 2, 10, and 13 months after the first intervention. Patients were followed a mean of 35 months after the first intervention. Results. Eight interventions were performed for diminished pulmonary blood flow and 1 intervention was performed for excessive pulmonary blood flow. Age at first intervention ranged from 28 to 48 years. Procedural success rate was 100%. There were no procedure‐related complications. Mean oxygen saturation increased from 77% prior to the procedure to 86% after the intervention. All patients experienced symptomatic improvement. There was 1 late sudden death 17 months post procedure. Conclusions. Percutaneous intervention can be performed safely and improves symptoms and oxygenation in adults with complex cyanotic heart disease.     

Unilateral Alveolar Consolidation with Uneven Pulmonary Artery Obstruction

Abstract: A case of unilateral alveolar consolidation associated with uneven pulmonary artery obstruction due to pulmonary thromboembolism is described in a 33-year-old woman presenting with advanced respiratory failure.     

Rehabilitation Program in Adult Congenital Heart Disease Patients with Pulmonary Hypertension

Introduction. It is commonly believed that physical activity may have a negative impact on pulmonary hypertension patients. The object of this study is to determine the tolerability of a directed exercise program in congenital heart disease patients with pulmonary hypertension. Methods. Eight congenital heart disease patients with pulmonary hypertension were studied and followed up during a 1-year period. Four of them were enrolled in a 3-month rehabilitation program. Results. No significant changes in analytical data, hand and leg strength, or quality of life were seen at the end of the training program in rehabilitation and nonrehabilitation patients. However, patients in the rehabilitation group improved 6 minutes' walk test minimum hemoglobin oxygen saturation and functional class after ending the training program without having adverse events such as progression of symptoms or heart failure. Conclusions. Cardiopulmonary rehabilitation appears to be a safe intervention in patients with congenital heart disease and pulmonary hypertension.     

Anomalous Left Coronary Artery from the Pulmonary Artery with a Large Patent Ductus Arteriosus: Aversion of a Catastrophe

We present an infant who had an anomalous left coronary artery arising from the pulmonary artery (ALCAPA) and a large patent ductus arteriosus (PDA), who was diagnosed before a potentially catastrophic closure of PDA. In the presence of normal left ventricular function and the absence of coronary artery collaterals, it is difficult to diagnose ALCAPA. A disproportionate degree of left ventriclular dilation and severity of mitral valve regurgitation relative to the degree of PDA shunt, and echogenic papillary muscles on an echocardiogram should raise a suspicion of coronary artery anomalies. The infant underwent surgical ligation of PDA with translocation of coronary arteries and had an uneventful recovery.     

Pulmonary Vein Stenosis with Down Syndrome: A Rare and Frequently Fatal Cause of Pulmonary Hypertension in Infants and Children

Down syndrome (DS) patients are prone to pulmonary hypertension (PHTN) due to various cardiopulmonary causes. However, the association of DS with pulmonary vein stenosis (PVS) is not adequately described. We illustrate three cases from our center and an additional 13 cases from an extensive review of the literature of DS patients with PHTN and PVS. In DS patients PVS is rare, they were diagnosed at a young age (<7.2 months), had high mean pulmonary artery pressures (38 mm Hg), and had rapid progression of the stenosis with an increased mortality (91%) in patients with two or more vein involvement. In DS patients, PVS may be missed by echocardiography; thus, any DS patients with persistent PHTN should undergo cardiac catheterization to assess hemodynamics and to evaluate all four pulmonary veins.     

The Impact of Pulmonary Venous Hypertension on the Pulmonary Circulation in the Young

Objective and Design. Pulmonary venous hypertension is a well‐characterized cause of pulmonary hypertension in adults, but little is known regarding the relationship between left atrial pressure and pulmonary arteriolar resistance in the young. Also, in adults relief of pulmonary venous hypertension results in a marked fall in pulmonary arteriolar resistance, but this could be different in children because vascular changes are more severe in young patients than adults with mitral stenosis. We inspected records of children at Children's Hospital Boston having mitral balloon valvuloplasty, and patients ≤5 years old having mitral valve replacement, to determine (1) the relationship between left atrial pressure and pulmonary arterial pressure and resistance (n = 94 children, median age 17.8 months) and (2) how pulmonary arteriolar resistance changes after mitral valve replacement. Results. The average indexed pulmonary arteriolar resistance was 7.8 ± 5.9 units and was unrelated to age but was positively related to left atrial pressure. There was great variability in pulmonary arteriolar resistance for any given left atrial pressure. Pulmonary arterial pressure (n = 16) and pulmonary arterial resistance (n = 9) were measured before and after mitral valve replacement (median = 29.4 months old). Despite preoperative indexed pulmonary arterial resistance of ≥5 units in 11 of 15 patients, postoperative pulmonary arterial pressure was substantially lower in all save three, and two patients with high pulmonary arterial pressure still had high left atrial pressure postoperatively (25 mmHg). Conclusions. We conclude that in young children, as in adults, pulmonary arterial resistance generally falls greatly with reduction in left atrial pressure.     

阿托伐他汀逆转早期野百合碱诱导肺动脉高压机制

目的本实验研究探讨,阿托伐他汀早期干预由脱氢野百合碱诱导肺动脉高压的比格犬的作用及其可能的机制。方法分组:阿托伐他汀组n=7(2mg/kg野百合碱+2mg/kg阿托伐他汀);野百合碱组n=5(2mg/kg野百合碱组+安慰剂);正常对照组n=6(溶剂0.1ml/kg二甲基酰胺组+安慰剂);药物干预前和干预8周后测量血流动力学参数,用免疫组织化学染色法检测肺小动脉半胱氨酸天冬氨酸蛋白酶-3(Caspase-3)、平滑肌肌动蛋白(α-smooth muscle actin,α-SMA)蛋白表达;用实时定量PCR法检测各组肺组织内皮型一氧化氮合酶(eNOS)、内皮素前体-1(preproET-1)、白介素-1β(IL-1β)、肿瘤坏死因子-α(TNF-α)、血管内皮生长因子(VEGF)mRNA表达。结果阿托伐他汀组犬的肺动脉压力,右心室收缩压,肺动脉收缩压,肺血管阻力较野百合碱组明显下降(P<0.05),而心输出量明显升高(P<0.05),并接近正常对照组。肺组织preproET-1mRNA、IL-1βmRNA、TNF-αmRNA、VEGF mRNA转录水平在阿托伐他汀组显著低于野百合碱组(P<0.05);eNOS ...