Suprasternal Approach to Transcatheter Aortic Valve Replacement in a Complex Congenital Pediatric Patient Presenting With Cardiogenic Shock.

Transcatheter aortic valve replacement (TAVR) offers well established benefit for adults with severe aortic stenosis, although applications in the pediatric population remain limited. We describe a case of a 15-year-old male with complex congenital heart disease presenting with cardiogenic shock in the setting of mixed severe aortic stenosis (AS) and severe aortic insufficiency (AI). Self-expanding TAVR was performed via suprasternal approach with robust clinical improvement.

Surgical Management of Heterotaxy Syndrome: Current Challenges and Opportunities.

Introduction: Heterotaxy syndrome presents a unique challenge in surgical management, even in the current era. We hypothesized that certain anatomic subsets merit novel strategies.

Methods: We analyzed morphologic details, surgeries, comorbidities, subsequent admissions, and survival using Kaplan-Meier methods and multivariable risk models from a single-institution experience of 103 consecutive patients with heterotaxy who underwent cardiac surgery between January 1, 1990, and May 31, 2016.

Extracorporeal membrane oxygenation support for hypertrophic cardiomyopathy in an infant of a diabetic mother.

Infants of diabetic mothers are at risk for transient hypertrophic cardiomyopathy. These infants are typically asymptomatic, but may develop signs of poor cardiac output from left ventricular outflow tract obstruction. This case illustrates the successful use of extracorporeal membrane oxygenation to support cardiac output in an infant of a diabetic mother with outflow tract obstruction until hypertrophy improved.

Late Left Ventricular Outflow Tract Obstruction Following the Rastelli Operation: Expectations Out to 20 Years.

Background: Consensus is lacking regarding the optimal operation for transposition, ventricular septal defect, and pulmonary stenosis.

Methods: Between 1968 and 2012, a total of 76 patients underwent the Rastelli procedure, with 52 mid- or long-term survivors. A bracketing analysis was used to estimate the likelihood of late left ventricular outflow tract obstruction (LVOTO).

Novel technique of valve-sparing aortic root replacement in two children younger than 3 years of age.

A novel technique of valve-sparing aortic root replacement was applied to 2 children younger than 3 years of age with Marfan syndrome and large aortic root aneurysms. Using elements of both the remodeling and reimplantation techniques, circumferential rings from a 20-mm to 22-mm polyester graft provide stabilization at the subannular and sinotubular levels, and bovine pericardial patches create pseudosinuses. Follow-up at 2 years in 1 patient and 7 months in a second patient revealed satisfactory valve function with stable aortic root size.

Constitutional H19 hypermethylation in a patient with isolated cardiac tumor.

Beckwith-Wiedemann syndrome (BWS) is clinically and molecularly very heterogenous. Molecular findings characteristic of BWS have been reported in individuals with no or few associated features. We report on a child with isolated cardiac tumor and a constitutional H19 hypermethylation with none of the features of BWS.

Is the "perfect Fontan" operation routinely achievable in the modern era?

Objective: In 1990, Fontan, Kirklin, and colleagues published equations for survival after the so-called "Perfect Fontan" operation. After 1988, we evolved a protocol using an internal or external polytetraflouroethylene tube of 16 to 19 millimetres diameter placed from the inferior caval vein to either the right or left pulmonary artery along with a bidirectional cava-pulmonary connection. The objective of this study was to test the hypothesis that a "perfect" outcome is routinely achievable in the current era when using a standardized surgical procedure.

Identification of serious congenital heart disease in neonates after initial hospital discharge.

Objective: Neonates with congenital heart disease (CHD) frequently require prompt intervention to improve outcomes and limit morbidities. We sought to determine the time to identification for infants with serious CHD admitted to the State of Alabama's neonatal cardiovascular center and to identify types of cardiac lesions associated with an earlier vs. later diagnosis.

Live/Real time three-dimensional transthoracic echocardiographic assessment of aortopulmonary window.

Aortopulmonary (AP) window is an extremely rare congenital anomaly involving a communication between the ascending aorta and pulmonary artery. Two-dimensional transthoracic echocardiography is traditionally used in the diagnosis but has limitations. We report the usefulness of three-dimensional transthoracic echocardiography in the assessment of AP window in two newborns studied by us.

Assessment of surgically unroofed coronary sinus by live/real time three-dimensional transthoracic echocardiography.

Both two-dimensional transthoracic echocardiography (2DTTE) and live/real time three-dimensional transthoracic echocardiography (3DTTE) were attempted in a 25-year-old morbidly obese female with total anomalous pulmonary venous return (TAPVR) into the coronary sinus (CS) in whom surgical unroofing of CS with patch closure of CS ostium was performed in infancy to redirect pulmonary venous flow into the left atrium (LA). The patient had become increasingly symptomatic over the past 1 year because of severe left-to-right shunting due to dehiscence of the patch used to close the CS ostium. Despite a poor acoustic window, 3DTTE was able to identify a communication between the LA and CS which resulted from surgical unroofing of the CS as well as flow signals moving into the right atrium from the CS.

Pulmonary aneurysms and intracardiac thrombi due to Behçet's disease in an African-American adolescent with oculocutaneous albinism.

Behçet's disease (BD) is a systemic vasculitis that may involve a variety of organs. We describe a girl with oculocutaneous albinism (OCA) who, on initial evaluation for popliteal artery occlusion, was noted to have multiple cardiac thrombi. She later developed hemoptysis, which was the result of bilateral pulmonary artery aneurysms.

Usefulness of live/real time three-dimensional transthoracic echocardiography in the assessment of atrioventricular septal defects.

We studied nine patients (five newborns and infants, two children, and two adults) with atrioventricular septal defects (four complete, one intermediate, and four partial) utilizing live/real time three-dimensional transthoracic echocardiography (3DTTE) and a 4-MHz matrix array transducer. In all patients, 3DTTE provided additional morphological and/or functional information as compared to standard two-dimensional transthoracic echocardiography (2DTTE). 3DTTE may be a useful supplement to 2DTTE in the assessment of atrioventricular septal defects.