Mid-Systolic Notching of the Pulmonary Valve Doppler Signal is Highly Associated with Pulmonary Hypertension.

Mid-systolic notching (MSN) of the pulmonary valve Doppler signal represents a reflected systolic pressure wave from the pulmonary vasculature and is often seen in pulmonary hypertension (PH). We hypothesize that MSN is associated with a higher pulmonary vascular resistance (PVR) and mean pulmonary artery pressure (mPAP), and a diagnosis of PH in pediatric patients. This was a retrospective study of patients ≤ 18 years who had an echocardiogram obtained ≤ 30 days before catheterization for suspected PH. MSN was defined as an indentation in the initial two thirds of the systolic Doppler signal. PH was defined as mPAP > 20 mmHg and PVR ≥ 3.0 Wu  m2. Subgroups (MSN vs. normal) were compared. Receiver operator characteristic determined a continuous variable's discriminatory ability for a diagnosis of PH. Reproducibility of MSN was assessed. In total, 90 patients (73 with congenital heart disease) were included, of which 36 had MSN and 54 were normal. MSN patients were more likely to have PH, and had significantly higher mPAP, PVR, and lower pulmonary stroke volume. The presence of MSN had good discriminatory ability for PH diagnosis. The presence of MSN had high specificity (96%) for PH, whereas sensitivity was lower (54%). Reproducibility was 100% for MSN. MSN is a simple, highly reproducible echocardiographic metric associated with higher mPAP and PVR. When present, there is a high likelihood a diagnosis of PH confirmed by catheterization. Incorporation of MSN into imaging protocols may be useful. MSN appears worthy of further investigation in pediatric patients with suspected PH.

Myocardial Ischemia and Anomalous Origin of the Right Coronary Artery From the Pulmonary Artery in the Adult: Management Implications and Follow-Up.

Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a very rare and potentially fatal congenital heart defect due to myocardial ischemia secondary to coronary steal phenomenon. We present a case of an adult presenting with a myocardial infarction who was found to have ARCAPA. Three-dimensional (3D) reconstructed computed tomography angiography (CTA) was utilized preoperatively for surgical planning. Surgical technique as well as pre- and postoperative 3D CTA are described and literature reviewed.

Hammock Mitral Valve Repair in Infancy: Operative Steps Toward a Customized Reconstruction After Preoperative Planning.

Hammock mitral valve (MV) repair is historically technically difficult with a guarded prognosis. Surgical experience is extremely limited and variable outcomes are reported. The perioperative strategy and technical details of hammock MV repair in an infant who presented with severe mitral stenosis are described and review of the existing literature was undertaken.

Variable Treatment Approaches for Extracorporeal Membrane Oxygenation Complications in Neonates: A Case Series.

Extracorporeal membrane oxygenation complications involving vascular injury or intracardiac thrombosis in neonates are rare. We present a series of unique complications and describe their variable treatments.

Use of Amplatzer vascular plugs and Amplatzer duct occluder II additional sizes for occlusion of patent ductus arteriosus: A multi-institutional study.

BACKGROUND: Variable patent ductus arteriosus (PDA) morphology and the need to close PDAs in small size patients has led physicians to use Amplatzer Vascular Plugs (AVP) and recently available Amplatzer Duct Occluder II - Additional Sizes (ADO II AS). The purpose of this study was to analyze the safety, efficacy, and complication rates of the ADO II AS and AVPs, specifically AVP II. METHODS: All patients undergoing PDA closure with an AVP or ADO II AS from 2011 to 2016 were included. Clinical, echocardiographic, and angiographic data were collected and reviewed. RESULTS: Four hundred and sixty-nine patients were included. Median age was 27 months (0.75-852) and the median weight was 11.4 kg (1-92). There were 51 patients ≤5 kg. Type A PDA was most common in 48% (n = 225), followed by type E (27.5%, n = 129), type D (13%, n = 61), type C (10.2%, n = 48), and type B (1.3%, n = 6). Devices included AVP II (n = 421), ADO II AS (n = 30), and AVP IV (n = 18), left pulmonary artery stenosis occurred in 4.3% (n = 20). One patient required surgery for severe stenosis; the remaining cases were mild and required no intervention. Device embolization occurred in 3 patients (10%) with the ADO II AS. Successful device closure was achieved in 98.9% of cases. CONCLUSIONS: The AVP II was highly effective for closing PDAs in smaller babies with varying morphologies and is safe when used in small sized patients with relatively low risk of complications. More studies are warranted to clarify the risks of ADO II AS.

Neonatal Myocardial Perfusion in Right Ventricle Dependent Coronary Circulation: Clinical Surrogates and Role of Troponin-I in Postoperative Management Following Systemic-to-Pulmonary Shunt Physiology.

Right ventricle dependent coronary circulation (RVDCC) in pulmonary atresia with intact ventricular septum (PA/IVS) is associated with significant mortality risk in the immediate post-operative period following the initial stage of surgical palliation. Prognosis remains guarded during the interstage period towards conversion to the superior cavopulmonary shunt physiology. Current literature is scarce regarding this specific patient population. Cardiac troponin-I is widely used as a marker of coronary ischemia in adults, but its use for routine monitoring of neonatal myocardial tissue injury due to supply/demand perfusion mismatch is, yet to be determined. We sought to evaluate the clinical correlation of cTnl perioperative use in a PA/IVS RVDCC case and assess its interplay with established clinical, echocardiographic, and laboratory variables in guiding a real-time (dynamic) management strategy following systemic-to-pulmonary shunt palliation.

Percutaneous pulmonary valve implantation with anomalous left anterior descending coronary artery.

Percutaneous pulmonary valve placement in patients with an anomalous coronary artery is rare and can be complicated by coronary artery compression. We report successful implantation of a percutaneous pulmonary valve in a patient with an anomalous left anterior descending artery.

Restoring Fetal Circulation as a Means of Bridging Treatment Prior to Surgical Repair of Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta with Persistent Pulmonary Hypertension of the Newborn.

Anomalous origin of one pulmonary artery from the ascending aorta is a rare congenital anomaly. Even more rarely reported is its presence in conjunction with persistent pulmonary hypertension of the newborn (PPHN). We present a case of a full-term infant, initially thought to have PPHN and later found to have anomalous origin of the right pulmonary artery from the ascending aorta. We discuss our management concept which included use of PgE1 infusion to restore fetal circulation prior to surgical treatment in this unique clinical scenario.

The Challenge of Hammock Mitral Valve During Infancy: Precise Preoperative Advanced Imaging and Three-Dimensional Modeling Augments Customized Operative Valve Reconstruction.

Hammock mitral valve (MV), also known as anomalous mitral arcade, is a rare congenital anomaly. We report a case of a 10-month-old child who presented with congestive heart failure and was found to have severe mitral stenosis (MS) secondary to a hammock MV anomaly. Detailed advanced imaging with cardiac MRI and three-dimensional (3D) echocardiography favorably navigated a customized valve-sparing surgical reconstruction of the congenitally abnormal MV. Repair of a hammock MV is technically difficult with a guarded prognosis. Surgical experience is extremely limited and variable outcomes are reported. A rare case of severe MS secondary to hammock MV deformity with successful surgical repair and review of worldwide literature are presented.