Transcatheter Intervention for Congenital Defects Involving the Great Vessels: JACC Review Topic of the Week.

Since the development of balloon angioplasty and balloon-expandable endovascular stent technology in the 1970s and 1980s, percutaneous transcatheter intervention has emerged as a mainstay of therapy for congenital heart disease (CHD) lesions throughout the systemic and pulmonary vascular beds. Congenital lesions of the great vessels, including the aorta, pulmonary arteries, and patent ductus arteriosus, are each amenable to transcatheter intervention throughout the lifespan, from neonate to adult. In many cases, on-label devices now exist to facilitate these therapies. In this review, we seek to describe the contemporary approach to and outcomes from transcatheter management of major CHD lesions of the great vessels, with a focus on coarctation of the aorta, single- or multiple-branch pulmonary artery stenoses, and persistent patent ductus arteriosus. We further comment on the future of transcatheter therapies for these CHD lesions.

Outcomes of Surgical Reimplantation for Anomalous Origin of One Pulmonary Artery From the Aorta.

BACKGROUND: Anomalous origin of one pulmonary artery from the ascending aorta (AOPA) is a rare and potentially deadly anomaly. Little research, aside from case reports on APOA, has been published, especially for patients with late referrals. METHODS: This study was a retrospective review of 57 patients with AOPA who underwent reimplantation of the pulmonary artery (PA) from 2009 to 2019. Two different reimplantation methods were used to correct the anomaly, including direct anastomosis in 36 patients and angioplasty with autologous tissue in 21 patients. RESULTS: The median age at repair was 2.8 months (range, 8 days to 3.6 years). In-hospital death occurred in 2 patients (3.5%). Five patients (9.1%) with a median age of 9.3 months (range, 5.2 months to 3.6 years) experienced a pulmonary hypertensive crisis. Patients older than 4.9 months were more likely to have a pulmonary hypertensive crisis (P = .001). The 2-year freedom from postoperative PA stenosis rate was 75.3% in patients who underwent direct anastomosis and 46.8% in patients who underwent autologous tissue angioplasty (χ2 = 4.878; P = .027). Angioplasty with autologous tissue (hazard ratio, 5.03; 95% confidence interval, 1.61 to 15.71; P = .005) and an innately smaller diameter of the aberrant PA (hazard ratio, 0.65; 95% confidence interval, 0.45 to 0.92; P = .015) were 2 independent risk factors for postoperative PA stenosis. CONCLUSIONS: Surgical reimplantation of the PA in patients with AOPA has resulted in favorable early and midterm outcomes. Pulmonary hypertensive crisis occurs more commonly in patients who receive a diagnosis after the age of 4.9 months. Reimplantation with autologous tissue augmentation and an intrinsically smaller diameter in affected PAs are 2 independent risk factors for postoperative PA stenosis.

Histology and Mechanics of In Vivo Tissue-Engineered Vascular Graft for Children.

PURPOSE: This study sought to evaluate the histologic and mechanical properties of autologous in vivo tissue-engineered vascular grafts (in vivo TEVGs) used for pediatric heart surgery. DESCRIPTION: Molds of in vivo TEVGs made of silicone drain tubes were embedded into subcutaneous spaces in 2 boys during their first operation and were used as patch materials to treat pulmonary artery stenosis during the second operation. The remaining pieces of the patches were evaluated histologically and mechanically. EVALUATION: In vivo TEVGs had very smooth luminal surfaces, and their walls mainly comprised collagen fibers and small numbers of fibroblasts. Mean wall thickness was 200 µm, mean suture retention strength was 2.26 N, and burst pressure was 3057 mm Hg. CONCLUSIONS: Human in vivo TEVGs mainly comprise collagen fibers, and their mechanical properties prove them safe for pulmonary arterioplasty. Therefore, human in vivo TEVGs may be promising alternatives to autologous pericardium for pediatric cardiovascular surgical procedures that often require multistage operations.

A review: Percutaneous pulmonary artery stenosis therapy: state-of-the-art and look to the future.

Stenosis, or narrowing, of the branches of the pulmonary artery is a type of CHD that, if left untreated, may lead to significant complications. Ideally, interventions to treat stenosis occur before significant complications or long-term sequelae take place, often within the first 2 years of life. Treatment depends on specifics of the condition, the presence of other malformations, and age of the child. Research and recent innovation to address these shortcomings have provided physicians with safer and more effective methods of treatment. This has further continued to push the ceiling of pulmonary arterial stenosis treatment available for patients. Despite continuous advancement in angioplasty - such as conventional and cutting balloon - and stenting, each treatment method is not without its unique limitations. New technological developments such as bioresorbable stents can accommodate patient growth and pulmonary artery stenosis treatment. As more than a decade has passed since the review by Bergersen and Lock, this article aims to provide a contemporary summary and investigation into the effectiveness of various therapeutic tools currently available, such as bare metal stents and potential innovations including bioresorbable stents.

Successful Infant Pneumonectomy with Unilateral Pulmonary Artery Occlusion Test.

The use of unilateral pulmonary artery occlusion (UPAO) test for the preoperative evaluation of pneumonectomy was reported in adult patients. On the contrary, in infants, no strategies have yet been recommended to predict hemodynamics after pneumonectomy, nor has use of the UPAO test been reported. We describe the first case of infant with abnormal pulmonary circulation in whom successful pneumonectomy was performed after preoperative evaluation using UPAO test. Right pneumonectomy was planned for an 8-month-old girl, because of decreased right pulmonary function, high risk of pneumothorax, and impaired left lung expansion due to overexpansion caused by severe left bronchial stenosis and bronchomalacia. However, she had also prolonged pulmonary hypertension and there was difficulty in accurate echocardiographic evaluation of its severity due to concomitant left pulmonary artery stenosis. Furthermore, contrast-enhanced computer tomography suggested a certain degree of right pulmonary venous flow, discordant with the result showing scarce right pulmonary flow in perfusion scintigraphy. Predicting postoperative hemodynamic changes was therefore considered difficult. To evaluate these concerns, we performed cardiac catheterization and UPAO test to simulate postoperative hemodynamics. Pulmonary arteriography showed decreased but significant right pulmonary arterial and venous flows. Measurements including pulmonary artery pressure and cardiac index showed no marked changes after occlusion. Based on UPAO test results, the operation was successfully performed and hemodynamics remained stable postoperatively. The UPAO test may be useful for infants with cardiopulmonary impairment to evaluate the tolerability of pneumonectomy.

Treatment of Severe Native Left Pulmonary Artery Stenosis With Coronary Stent Implantation in a 2.4 kg Neonate.

A 1-month-old, 2.4 kg infant, previously born at 32 weeks gestation, was found to have a murmur while in the neonatal intensive care unit. The patient had ongoing feeding intolerance and required supplemental oxygen via nasal cannula. Cardiac computed tomography showed discrete stenosis of the proximal left pulmonary artery (LPA) with a normal-sized distal LPA. We describe the treatment course with transcatheter coronary stent implantation.

Dehiscence of a pulmonary bioprosthesis with a focal dissection of the pulmonary artery in a patient with congenital pulmonic stenosis.

Pulmonary valve replacement (PVR) is the most common adult congenital cardiac operation performed. Valve degeneration leading to prosthetic stenosis and/or regurgitation is a long-term risk in this population and may be associated with paravalvular leak (PVL). Complications involving the proximal pulmonary artery, including dissection, are less clearly defined. Herein, we report the case of a 30-year-old patient with a history of multiple pulmonary valve interventions secondary to congenital pulmonic stenosis, who developed dehiscence of a bioprosthetic PVR associated with significant paravalvular leak (PVL) and further complicated by a focal dissection of the proximal pulmonary artery.