Utility of Three-Dimensional Printed Model in Biventricular Repair of Complex Congenital Cardiac Defects: Case Report and Review of Literature.

Heterotaxy is a rare syndrome associated with cardiac complexity, anatomic variability and high morbidity and mortality. It is often challenging to visualize and provide an accurate diagnosis of the cardiac anatomy prior to surgery with the use of conventional imaging techniques. We report a unique case demonstrating how the use of three-dimensional (3D) cardiac printed model allowed us to better understand the anatomical complexity and plan a tailored surgical approach for successful biventricular repair in a patient with heterotaxy syndrome.

Is Interarterial Course a Requisite for Ischemia in Anomalous Origin of Coronary Arteries?

Anomalous origin of the left main coronary artery from the noncoronary sinus (LCANS) is a rare variant of anomalous aortic origin of a coronary artery that is not characterized by an interarterial course. Despite the absence of an interarterial course, there are rare reports of LCANS presenting with sudden death, life-threatening arrhythmia, exercise-induced myocardial ischemia, premature ventricular contractions, and syncope. We report a patient with LCANS presenting with acute myocardial infarction who recovered completely with emergent surgical unroofing of the coronary artery.

Surgical management of secondary tricuspid valve regurgitation: annulus, commissure, or leaflet procedure?

OBJECTIVES: Techniques employed today concomitantly with left-sided heart valve surgery address secondary tricuspid valve regurgitation at 3 anatomic levels-annulus, commissure, and leaflet-although success of these alone or in combination in eliminating tricuspid regurgitation is uncertain. Our objective was to assess the comparative effectiveness of these techniques in reducing or eliminating secondary tricuspid regurgitation. METHODS: From 1990 to 2008, 2277 patients underwent tricuspid valve procedures for secondary tricuspid regurgitation concomitantly with mitral (n = 1527, 67%), aortic (n = 180, 7.9%), or combined (n = 570, 25%) valve surgery. These included annulus (flexible prosthesis [n = 1052, 46%], rigid prosthesis [standard = 387, 3-dimensional = 197; 26%], Peri-Guard annuloplasty [Synovis Surgical Innovations, St Paul, Minn; n = 185, 8.1%], and De Vega suture [n = 129, 5.7%]), commissure (Kay [n = 248, 11%]), and leaflet (edge-to-edge suture [n = 79, 3.5%] +/- annulus or commissural) procedures. A total of 4745 postoperative transthoracic echocardiograms in 1965 patients were analyzed. RESULTS: By 3 months after surgery, only 32% of patients overall had no tricuspid regurgitation. However, by 5 years, this had decreased to 22%, and 3+/4+ tricuspid regurgitation had increased from 11% at 3 months to 17%. Patients with rigid ring annuloplasty alone, either standard or 3-dimensional, had the least increase of 3+/4+ tricuspid regurgitation (to 12% at 5 years) compared with either a commissural or leaflet procedure. CONCLUSION: Rigid prosthetic ring annuloplasty, standard or 3-dimensional, provides early and sustained reduction of tricuspid regurgitation secondary to left-sided valve disease without need for an additional leaflet procedure. However, results are imperfect, possibly because other anatomic levels (subvalvular, papillary muscle, and right ventricular) contributing to its pathophysiology are unaddressed.

Aortic translocation for the management of double-outlet right ventricle and pulmonary stenosis with dextrocardia: technique to avoid coronary insufficiency.

The management of D-loop transposition of the great arteries with left ventricular outflow tract obstruction and related forms of double-outlet right ventricle is challenging. Association with right ventricular and tricuspid valve hypoplasia and dextrocardia represents a major obstacle to achieve a biventricular repair. The most commonly used technique to deal with this condition, which is the Rastelli operation, further reduces the potentially compromised right ventricular volume due to the left ventricle-to-aorta baffle. Because the Rastelli operation risks later development of left ventricular outflow tract obstruction, aortic translocation can be considered for these patients. We report a case of double-outlet right ventricle with left ventricular outflow tract obstruction in the setting of dextrocardia, left juxtaposition of the atrial appendages, and a small tricuspid valve, which was successfully managed with a biventricular repair by means of an aortic translocation technique.

Preoperative extracorporeal membrane oxygenation as a bridge to cardiac surgery in children with congenital heart disease.

BACKGROUND: The efficacy of extracorporeal membrane oxygenation (ECMO) in bridging children with unrepaired heart defects to a definitive or palliative surgical procedure has been rarely reported. The goal of this study is to report our institutional experience with ECMO used to provide preoperative stabilization after acute cardiac or respiratory failure in patients with congenital heart disease before cardiac surgery. METHODS: A retrospective review of the ECMO database at Children's Hospital Boston was undertaken. Children with unrepaired congenital heart disease supported with ECMO for acute cardiac or respiratory failure as bridge to a definitive or palliative cardiac surgical procedure were identified. Data collection included patient demographics, indication for ECMO, details regarding ECMO course and complications, and survival to hospital discharge. RESULTS: Twenty-six patients (18 male, 8 female) with congenital heart disease were bridged to surgical palliation or anatomic repair with ECMO. Median age and weight at ECMO cannulation were, respectively, 0.12 months (range, 0 to 193) and 4 kg (range, 1.8 to 67 kg). Sixteen patients (62%) survived to hospital discharge. Variables associated with mortality included inability to decannulate from ECMO after surgery (p = 0.02) and longer total duration of ECMO (p = 0.02). No difference in outcomes was found between patients with single and biventricular anatomy. CONCLUSIONS: Extracorporeal membrane oxygenation, used as a bridge to surgery, represents a useful modality to rescue patients with failing circulation and unrepaired complex heart defects.

Pattern of pulmonary venous flow in patients with ostium secundum atrial septal defect.

AIMS: The purpose of this study is to determine the possible influence of a left to right atrial shunt over the pulmonary venous flow pattern in children with ostium secundum atrial septal defect (ASD). METHODS AND RESULTS: Complete two-dimensional, spectral Doppler and colour flow Doppler examination were undertaken to examine the pulmonary vein flow pattern in 74 patients with ASD. This group was compared with a control group of 49 non-cardiological patients of similar ages and gender. Ultimately, pulmonary venous flow patterns changed. Pulmonary vein systolic velocities were significantly increased and diastolic velocities decreased in the ASD patient group compared with the control group. The right ventricular (RV) systolic function, evaluated by tricuspid annular displacement and tricuspid annular systolic velocity, was increased in patients with ASD. No left ventricular dysfunction was detected in the patient study group. CONCLUSION: Patients with ASD show changes in the pattern of pulmonary venous flow with an increase in systolic velocity and a decrease in diastolic velocities. This could be caused by an increase in the RV systolic function, which creates a vacuum effect against not only blood from the superior and inferior vena cavae, but also blood from the pulmonary vein through the ASD.

[Echocardiographic Doppler evaluation of ventricular function in children with an atrial septal defect]. / Evaluación de la función ventricular por ecocardiografía Doppler en niños con comunicación interauricular.

INTRODUCTION AND OBJECTIVES: To evaluate ventricular function using Doppler echocardiography in patients with an atrial septal defect (ASD). METHODS: The study involved 158 children, who were divided into two groups: Group 1 comprised 111 children with an ASD, and Group 2, 47 healthy children. Left ventricular systolic function was evaluated by measuring the ejection fraction, the shortening fraction, and the systolic mitral annular velocity (by tissue Doppler), and right ventricular systolic function, by measuring the systolic tricuspid annular velocity. Left ventricular diastolic function was evaluated by measuring standard transmitral Doppler indices, the color M-mode Doppler flow propagation velocity (Vp), the E/Vp ratio, and early and late diastolic mitral annular velocities at the lateral and septal areas of the mitral annulus (EML, EMS and AML, AML waves, respectively), and right ventricular diastolic function, by using pulsed tissue Doppler to measure early and late diastolic tricuspid annular velocities (ET and AT waves, respectively). RESULTS: There was no significant difference between the two groups in any left ventricular systolic or diastolic function parameter, except that the EML and EMS velocities were smaller in Group 1. Right ventricular systolic function was significantly increased in children with an ASD. The peak ET wave velocity was similar in the two groups but the peak AT wave velocity was significantly greater in children with an ASD. CONCLUSIONS: Systolic and diastolic left ventricular function (i.e., preload-independent indices) were similar in the two groups. Children with an ASD demonstrated an increase in right ventricular systolic function, though diastolic function was unchanged.