Large Apical Muscular Ventricular Septal Defect: Asymptomatic due to Anomalous Muscle Bundles in the Right Ventricle

This case report demonstrated an apical muscular ventricular septal defect (VSD) that was a large defect but behaved like a small defect because of the restrictive flow across the anomalous muscle bundles in the right ventricular (RV) apex. The anomalous muscle bundles separated the RV sinus into two parts: the RV apex connecting with the left ventricle through the apical muscular VSD on one side, and the rest of the RV sinus connecting with RV inflow and RV outflow on the other side. These findings explained why the 11-year-old girl in this study remained asymptomatic without evidence of volume load. Thus far, it was not necessary to close her defect because of the hemodynamic insignificance.     

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.     

No Inflammatory Response Related to Pulmonary Hemodynamics in Children with Systemic to Pulmonary Shunts

Objective. The hypothesis was that the levels of circulating inflammatory mediators are related to the degree of volume and pressure stress on the pulmonary vasculature in children with congenital systemic to pulmonary shunts. Design. Prospective, cross‐sectional study. Setting. Tertiary center covering all pediatric heart surgery and interventions in Norway. Patients. Seventy‐four children, aged 0–12 years, admitted for surgical or interventional treatment of congenital systemic to pulmonary shunts. Outcome Measures. Plasma levels of eight mediators of vascular inflammation and endothelial activation, sampled from different vascular compartments. Results. Patients with the most pronounced pulmonary flow and pressure stress demonstrated no elevation of inflammatory mediator levels when compared with healthy controls. No pulmonary production or uptake of the measured markers was found. Hemodynamic explanatory factors showed weak correlations to the inflammatory marker levels by univariate analysis. Age was the only factor that significantly explained inflammatory response in the multivariate model. The presence of Down syndrome, irrespective of hemodynamic category, was associated with elevated plasma levels of soluble tumor necrosis factor receptor I, when controlling for age. Conclusions. Inflammatory mediators show no significant relationship to pulmonary hemodynamics in children with systemic to pulmonary shunts. Children with Down syndrome may have an increased inflammatory response.     

Right Ventricular Endocarditis in a Pregnant Woman with a Restrictive Ventricular Septal Defect

A 22‐year‐old woman with a restrictive unoperated perimembranous ventricular septal defect was diagnosed with staphylococcal endocarditis during her 14th week of pregnancy. Echocardiography revealed a long, thin, and mobile vegetation along the right ventricular free wall that increased to 8 cm in length, with systolic protrusion across the pulmonary valve. The vegetation subsequently embolized, resulting in a pulmonary abscess. She responded favorably to intravenous antibiotic therapy maintained for a total of 6 weeks, with resolution of the intracardiac mass and pulmonary abscess. The remaining peripartum and postpartum course was relatively unremarkable. Percutaneous closure of the ventricular septal defect was successfully performed postpartum.     

Atrial Septal Stent Implant: Atrial Septal Defect Creation in the Management of Complex Congenital Heart Defects in Infants

Objectives. Certain congenital heart defects require the creation of an unrestrictive atrial septal defect (ASD) to relieve atrial hypertension, to maintain systemic cardiac output, or to achieve adequate atrial mixing to improve systemic oxygen saturation. We describe a series of patients, ranging in age from 5 weeks to 17 months, in whom we implanted a stent across the atrial septum to create a lasting, unrestrictive interatrial communication. Methods. Five patients with left heart obstructive defects presented with severe left atrial hypertension. The patients weighed 3.5–10.1 kg. Patients 1 and 2 presented at 5 and 9 weeks of age, respectively. Patient 1 had a restrictive patent foramen ovale, and patient 2 had an intact atrial septum. Patients 3, 4, and 5 had previous cardiac surgery including atrial septectomy. At presentation, all patients were poor surgical candidates. Results. Using standard percutaneous technique, a premounted Palmaz–Genesis stent (Cordis Corporation) was implanted across the atrial septum in all 5 patients, using balloons ranging from 7 to 10 mm diameter. Each patient had a dramatic decrease in left atrial pressure and increased ASD diameter. All stented ASDs remained widely patent, confirmed by Doppler echocardiography, until elective surgical stent explant. Conclusion. Patients presenting beyond the neonatal period with left heart obstructive defects and intact atrial septum or restrictive ASD present a challenging problem. We report a safe and effective technique for relief of left atrial hypertension in infants using premounted intravascular stents. All patients had immediate marked hemodynamic improvement.     

Long‐term Follow‐up of “Simple” Lesions—Atrial Septal Defect,Ventricular Septal Defect,and Coarctation of the Aorta

Surgery for congenital heart disease has advanced significantly in the past 50 years, such that repair of “simple” lesions, such as atrial septal defect, ventricular septal defect, and coarctation of the aorta carries minimal risk, with mortality risk much less than 1%. It was once thought successful repair of these lesions was definitively corrective. There is mounting evidence, however, that there are long‐term complications after these repairs, prompting the need for continued follow‐up. This review describes the current understanding of diagnosis, treatment, and long‐term outcomes for these patients, with the goal of advocating for lifelong surveillance. As the perioperative care of these repairs has evolved significantly over time, so must the way in which we study these patients in the long‐term.     

Association of Left Ventricular Diverticula and Sinus Venosus Atrial Septal Defect

Left ventricular diverticula are congenital anomalies and are not as rare as previously thought. In adults, cardiac diverticula are generally accidental findings during imaging modalities, but concomitant disorders might frequently coexist. The pathophysiology, management, prognosis, and natural history of cardiac diverticula remain poorly understood and controversial. Definite diagnosis is often challenging due to similarities in appearance to other more common anomalies such as aneurysms, pseudoaneurysms, endocarditis, cysts, and hypertrophied trabeculations. We herein report a rare case of an adolescent presenting with sinus venosus atrial septal defect, partial anomalous venous connection, and left ventricular diverticula.     

Surgical Treatment of 144 Children with Ventricular Septal Defect and Severe Pulmonary Hypertension

Objective To report the result of surgical treatment for 144 children with ventricular septal defect and severe pulmonary hypertension. Method The diagnosis had been confirmed by cardiac catheter before the operation, Pp/Ps (0. 81±0.09) and Rp (9.41 ±5.37 ) wood. Ventricular septal prothesis was performed under cardiopulmonary bypass. Transthoracic intracardiac monitoring line inserted into pulmonary artery in 117 cases. Results Fourteen cases died of the 144 postoperatively, with mortality of 9.12% . Preoperative Pp/Ps≥0. 8 and Rp≥10 wood made the mortality higher than that of others ( P < 0. 05 ) . 32 persons had post - operative SpPA > 50 mmHg among the survivors and among the children with preoperative Pp/Ps ≥0. 8 and Rp≥10 wood the mortality was 28. 6 % being higher than that among those with Pp/Ps≥0. 8 and Rp < 10 wood. Conclusion The higher the preoperative Pp/Ps and Rp are, the higher the surgical mortality is. The prevention and treatment of postoperative pulmonary hypertension crisis sh     

Patients with Pulmonary Hypertension Related to Congenital Systemic‐to‐Pulmonary Shunts are Characterized by Inflammation Involving Endothelial Cell Activation and Platelet‐mediated Inflammation

Objective. We examined inflammatory mediators in patients with pulmonary hypertension related to congenital systemic‐to‐pulmonary shunts and the change in these markers during treatment with bosentan. Background. Inflammatory mechanisms probably play a pathogenic role in idiopathic pulmonary arterial hypertension. Their involvement in pulmonary hypertension related to congenital systemic‐to‐pulmonary shunts is largely unknown. Patients and Methods. Plasma levels of several inflammatory mediators were determined by enzyme immunoassays in 14 children and adolescents with pulmonary hypertension related to congenital systemic‐to‐pulmonary shunts before and after 12 months treatment with bosentan, and compared with levels in 54 healthy controls. Results. The patients were characterized by increased plasma levels of von Willebrand factor (～2.5‐fold), C‐reactive protein (～3.5‐fold), and soluble CD40 ligand (～2.5‐fold) as compared with controls, representing markers of endothelial cell activation, systemic inflammation, and platelet‐mediated inflammation, respectively. Patients also had significantly elevated plasma levels of osteoprotegerin (～1.6‐fold). Within the study group, N‐terminal pro‐brain natriuretic peptide levels correlated significantly with the concentrations of C‐reactive protein (r= 0.61, P < .027) and von Willebrand factor (r= 0.74, P= .004). Except for a decline in monocyte chemoattractant protein‐1 and receptor activator of nuclear factor‐κB ligand, bosentan therapy did not attenuate the systemic inflammation. Conclusion. Children and adolescents with pulmonary hypertension related to congenital systemic‐to‐pulmonary shunts are characterized by enhanced systemic inflammation involving increased endothelial cell activation and platelet‐mediated inflammation. These inflammatory responses seem essentially to be unmodified by bosentan, potentially representing new targets for therapy in this disorder.     

Evaluation of Pulmonary Artery Banding in the Setting of Ventricular Septal Defects and Severely Elevated Pulmonary Vascular Resistance

Background. Children with pulmonary hypertension secondary to large left‐to‐right, post‐tricuspid valve shunts can eventually have severe and/or irreversible pulmonary vascular disease, yielding them inoperable for conventional surgery. It has been shown, however, that unloading of the pulmonary hypertension can result in remodeling of the pulmonary vasculature and, thus, improvement of the pulmonary hypertension. Methods. This study explored whether such patients might experience a significant reduction in pulmonary vascular resistance (PVR) after pulmonary artery band (PAB) placement. Pulmonary hypertension hemodynamics were evaluated by cardiac catheterization in 4 patients with pulmonary hypertension secondary to nonrestrictive left‐to‐right, post‐tricuspid valve shunts before and after PAB placement. Two patients with severe pulmonary hypertension who were considered high risk for conventional surgery benefited from PAB placement with a significant reduction in their PVR, permitting subsequent complete intracardiac repair. Results. The medium‐term follow‐up for these 2 patients demonstrated good outcomes. The PVR failed to improve after PAB placement in the remaining 2 patients, leading to medical therapy for pulmonary hypertension. There was 1 late death, presumably related to pulmonary hypertension. Current practice provides 3 relatively unattractive options for patients with severe pulmonary hypertension secondary to nonrestrictive left‐to‐right, post‐tricuspid valve shunts: transplantation, high‐risk intracardiac repair, or palliative medical therapy. Conclusion. Our study suggests that a staged approach with initial PAB placement can be considered for select patients with large left‐to‐right, post‐tricuspid valve shunts and high PVR prior to committing them to other high‐risk therapeutic options.     

Right Aortic Coarctation and Ventricular Septal Defect: An Unusual Cause of Tracheal Compression in Infancy

Objective. Acyanotic congenital heart diseases may occasionally present with tracheobronchial obstruction. Increased pulmonary blood flow against a high‐resistance pulmonary bed may create significant pulmonary artery dilation. Methods. We report an unusual case of ventricular septal defect and right aortic arch coarctation, complicated with distal tracheal compression secondary to a pincer effect created by a right aortic arch and a massively dilated pulmonary artery. Results. High index of suspicion is required to anticipate tracheobronchial compression in acyanotic congenital heart diseases. Conclusion. Fiberoptic bronchoscopy is an invaluable tool to assess for tracheal compression relief following surgical repair and to identify tracheomalacia prior to extubation.     

Imaging of Extralobar Pulmonary Sequestration in a Patient with Tetralogy of Fallot with Pulmonary Atresia,Ventricular Septal Defect and Multiple Aorto‐pulmonary Collateral Arteries with Multidetector Computed Tomography

Complex pulmonary vascular blood supply is common in patients with tetralogy of Fallot with pulmonary atresia, major systemic to pulmonary collateral arteries and hypoplastic or deficient central pulmonary arteries. An extralobar lung sequestration, which has not been described previously in these patients, was imaged in a 6‐week‐old infant with multidetector computed tomography with sub‐millimeter resolution. Arterial and venous vessels were analyzed using three‐dimensional vascular exploration tools and results were confirmed with cardiac catheterization.     

前列地尔注射液对室间隔缺损介入治疗后血浆氨基末端脑利钠肽前体水平及心功能的影响

目的 观察先天性心脏病室间隔缺损（VSD）患儿介入封堵术后联合应用前列地尔注射液对术后血浆氨基末端脑利钠肽前体（NT-proBNP）水平及心功能的影响。方法 选择本院2011年1月至2013年1月住院行介入封堵治疗的VSD患儿120例,男51例,女69例,年龄5～8,平均6.1±2.3岁。随机分为前列地尔组60例,对照组60例,前列地尔组在封堵术后即刻注射前列地尔注射液连续7天,分别在术前、术后第3天、术后6个月采用双向侧流免疫法测定其血浆NT-proBNP水平,所有患儿在术前、术后第7天和术后6个月分别进行超声心动图检查,测定左心室舒张期末内径（LVEDD）、左心室收缩期末内径（LVESD）、左心室收缩期末容量（LVESV）、左心室舒张期末容量（LVEDV）、左心室射血分数（LVEF）,并比较两组临床特征、血浆NT-proBNP水平及心功能参数的变化。结果 两组患儿LVEDD、LVESD、LVEDV、LVESV在介入术后第7天及术后6个月均明显小于术前（P<0.05）,且封堵术后6个月前列地尔组LVEDD、LVESD、LVEDV、LVESV较对照组减小更明显（P<0.05）。两组患儿术前血浆NT-proBNP水平无统计学差异（P>0.05）;两组患儿血浆NT-proBNP水平术前与术后第3天、术后第3天与术后6个月比较差异均有统计学意义（P<0.01）,且前列地尔组封堵术后3天、术后6个月较对照组下降更明显（P<0.01）。结论 VSD患儿介入封堵术联合前列地尔治疗可进一步降低血浆NT-proBNP水平并改善患儿心功能。