Leadless Pacemaker Implantation in Fontan Patients with Multimodality Imaging: Tips and Tricks.

Current leadless pacemaker (LP) systems, which have been developed and used in patients with normal cardiac anatomy, are rare and technically even more challenging to implant in patients with congenital heart diseases, especially with univentricular physiology and Fontan palliation. We report two cases of percutaneous LP implantation in an adult and a child, respectively, highlighting the unconventional approaches, different challenges, and use of multimodality imaging in patients who underwent a Fontan operation.

Cardiac magnetic resonance assessment of acute rejection and cardiac allograft vasculopathy in pediatric heart transplant.

Background: In pediatric heart transplant (PHT), cardiac catheterization with endomyocardial biopsy (EMB) is standard for diagnosing acute rejection (AR) and cardiac allograft vasculopathy (CAV) but is costly and invasive.

Objectives: To evaluate the ability of cardiac magnetic resonance (CMR) to noninvasively identify differences in PHT patients with AR and CAV.

Methods: Patients were enrolled at three children's hospitals.

Cardiac magnetic resonance diastolic indices correlate with ventricular filling pressures in pediatric heart transplant recipients.

Background: Atrial and ventricular filling pressures are routinely used in pediatric heart transplant (PHTx) recipients to assess graft function. We hypothesized that cardiac magnetic resonance (CMR) diastolic indices correlate with filling pressures, providing a noninvasive method of hemodynamic assessment.

Methods: Pediatric heart transplant recipients were prospectively enrolled at the time of cardiac catheterization.

Dilatable Pulmonary Artery Banding Palliation in Congenital Heart Disease.

Background: Surgical pulmonary artery banding (PAB) has been limited in practice because of later requirement for surgical removal or adjustment. The aim of this study is to describe our experience creating a dilatable PAB via transcatheter balloon dilation (TCBD) in congenital heart disease (CHD) patients.

Methods: Retrospective chart review of adjustable PAB-outline anatomical variants palliated and patient outcomes.

The Destiny of Postoperative Residual Ventricular Septal Defects After Surgical Repair in Infants and Children.

Purpose: Residual ventricular septal defects (rVSDs) of small size are commonly seen on transesophageal echocardiography after surgical repair. This study aimed to determine the destiny of rVSD found on intraoperative echocardiogram.

Methods: Patients undergoing surgical repair of VSD as the primary procedure with available intraoperative and discharge echocardiograms between 2007 and 2017 were reviewed.

Correlation of gene expression profiling score, cardiac hemodynamics and echocardiographic parameters in asymptomatic, rejection-free pediatric heart transplant recipients.

Objectives: To correlate gene expression profiling scores obtained by AlloMap with cardiac hemodynamics, cardiac allograft vasculopathy (CAV), and echocardiographic parameters in asymptomatic, rejection-free pediatric heart transplant (HT) recipients.

Methods: Single-institution retrospective study of 210 AlloMap scores obtained concomitantly with cardiac catheterization and echocardiogram from 55 children during follow-up after cardiac transplantation.

Results: The median age at HT was 5.

Senning Procedure for Physiological Atrial Inversion With Left Atrial Isomerism.

We present the case of an infant with left atrial isomerism with complex pulmonary and systemic venous connections that resulted in physiological parallel circulation in the setting of ventriculoarterial concordance who was surgically treated using the Senning procedure. This case highlights a rare cause for cyanosis due to poor mixing from a parallel circulation and an issue with nomenclature. ().

Syncope in a Child with Pulmonary Hypertension and Positive Gene Tests for Hereditary Hemorrhagic Telangiectasia and Long QT Syndrome.

We present a 10-year-old boy with syncope who was found to have long-QT syndrome and severe Pulmonary Hypertension (PH) both in the absence of a secondary cause; to our knowledge, this is the first report with this unusual coexistence. His genetic tests were positive for hereditary hemorrhagic telangiectasia and Long QT Syndrome (LQTS) without any family history of PH or LQTS. We demonstrated that digital subtraction pulmonary angiography was more useful compared to CT angiogram to demonstrate pulmonary vascular changes which correlated with a noresponse to acute vasoreactivity testing during right heart catheterization.

Pulmonary Vascular Underperfusion Score in Premature Infants with Bronchopulmonary Dysplasia and Pulmonary Hypertension.

Pulmonary hypertension (PH) is a complication of bronchopulmonary dysplasia (BPD). The underlying pathophysiology of BPD-associated PH is complex and poorly understood. Echocardiogram may underestimate the severity of pulmonary hypertensive vascular disease in severe BPD.

Digital Subtraction Pulmonary Angiography in Children with Pulmonary Hypertension due to Bronchopulmonary Dysplasia.

Bronchopulmonary dysplasia (BPD) is the most common respiratory sequelae of prematurity and histopathologically features fewer, dysmorphic, pulmonary arteries. We present our experience with the digital subtraction pulmonary angiography (DSPA) findings of a segmental vascular filling abnormality in three children who were born at extreme prematurity and have pulmonary hypertension due to severe BPD. Our preliminary data suggest that DSPA may be useful in evaluating the severity of pulmonary vascular disease in children with BPD.

Acquired and congenital coronary artery abnormalities.

Sudden unexpected cardiac deaths in approximately 20% of young athletes are due to acquired or congenital coronary artery abnormalities. Kawasaki disease is the leading cause for acquired coronary artery abnormalities, which can cause late coronary artery sequelae including aneurysms, stenosis, and thrombosis, leading to myocardial ischaemia and ventricular fibrillation. Patients with anomalous left coronary artery from the pulmonary artery can develop adequate collateral circulation from the right coronary artery in the newborn period, which remains asymptomatic only to manifest in adulthood with myocardial ischaemia, ventricular arrhythmias, and sudden death.

Takotsubo cardiomyopathy: an unusual cardiomyopathy at an unusual age.

We describe an atypical presentation of stress-induced cardiomyopathy - Takotsubo cardiomyopathy - in a 16-month-old boy previously diagnosed with cyclic vomiting and episodic hypertension. He developed features of cardiac failure and his echocardiogram showed left ventricular wall motion abnormality accompanied with elevated cardiac enzymes. Cardiac catheterisation showed no coronary arterial abnormality.

Preliminary experience with cardiac reconstruction using decellularized porcine extracellular matrix scaffold: human applications in congenital heart disease.

An ideal material for repair of congenitally malformed hearts would encourage tissue regeneration with growth potential. Decellularized porcine small intestinal submucosa extracellular matrix (SIS-ECM) promotes tissue regeneration in animal models and noncardiac human applications. This retrospective review evaluates SIS-ECM for reconstruction of congenital heart defects.

Long-term outcome of palliation with internal pulmonary artery bands after primary heart transplantation for hypoplastic left heart syndrome.

The purpose of this study was to describe the long-term outcome of infants with hypoplastic left heart syndrome (HLHS) who underwent placement of internal pulmonary artery bands as part of a transcatheter palliation procedure followed by primary heart transplantation. Transcatheter palliation included stenting of the ductus arteriosus, decompression of the left atrium by atrial septostomy, and internal pulmonary artery band placement. Cardiac hemodynamics, pulmonary artery architecture, and pulmonary artery growth since transplantation are described.

Sildenafil Increases Systemic Saturation and Reduces Pulmonary Artery Pressure in Patients with Failing Fontan Physiology.

OBJECTIVE: The purpose of this study was to investigate the effect of sildenafil in patients with failing Fontan physiology. DESIGN: A retrospective chart review was performed to compare history and available data in patients with Fontan circulations before and after starting sildenafil. The paired and unpaired Student's t-tests were used for statistical analyses.

Interventional cardiac catheterization procedures in pediatric cardiac transplant patients: transplant surgery is not the end of the road.

Objectives: The objectives of this study are to report the spectrum of cardiac lesions in pediatric patients post-orthotopic heart transplantation (OHT), the characteristics of patients who develop these lesions, and the feasibility of transcatheter intervention in treating these lesions.

Background: Indications for OHT in the pediatric population range from cardiomyopathy to complex congenital heart defects with and without prior palliation. These patients may have residual vascular access and great vessel abnormalities.

Aortic pseudo aneurysm complicating coarctation stenting successfully treated with a PTFE balloon expandable covered stent.

Percutaneous stenting of both native and recurrent coarctation of the aorta has become an acceptable alternative to surgical repair in most centers throughout the world. Severe complications such as aortic rupture, dissection, and late pseudo aneurysm formation are rare but worrisome complications. In many countries, commercially available balloon expandable covered stents designed for intravascular use are used either for primary stenting, or in treating complications once identified.

The design and deployment of the HELEX septal occluder.

The GORE HELEX Septal Occluder (W.L. Gore and Associates, Flagstaff, Ariz, USA) is the latest device to pursue U.

On-line multi-slice computed tomography interactive overlay with conventional X-ray: a new and advanced imaging fusion concept.

Background: Computed tomography (CT) has revolutionized noninvasive cardiovascular evaluations. Complicated percutaneous procedures require precise imaging guidance that conventional X-ray is often unable to provide. By combining X-ray imaging with real-time, interactive, CT-based landmarks, interventional procedures could be facilitated.

Percutaneous selective pulmonary artery bands (Joeys) in a pulmonary overcirculation model.

Background: Control of distal pulmonary artery (PA) pressure and flow is a critical step in palliating infants with complex congential heart disease. Surgical procedures to protect or isolate the pulmonary circulation carry significant risk and can be unpredictable. Interventional control of pulmonary pressure/flow with an intravascular device (band) could reduce risk and improve regulation of pulmonary flow.

Percutaneous closure of a para-prosthetic aorto-right ventricular fistula.

A 60-year-old woman with increasing dyspnea was found to have a para-prosthetic aorto-right ventricular fistula during post-operative evaluation. Due to her multiple sternotomies, she was considered to be high risk for open surgical repair and referred for percutaneous intervention. A 6/4 mm Amplatzer occluder device (AGA Medical, Golden Valley, MN) was successfully delivered across the fistula with excellent immediate angiographic results.

Birth weight and complexity are significant factors for the management of hypoplastic left heart syndrome.

Background: Classic options for treatment of hypoplastic left heart syndrome include the Norwood procedure (NW) and heart transplantation (HT). Recently off-pump palliative procedures were introduced in the management of these patients. Risk factors influencing the decision between the NW with staged reconstruction or off-pump palliation and HT were assessed.