Humberto Restrepo

Prenatal Detection of Congenital Heart Disease in Southern Nevada The Need for Universal Fetal Cardiac Evaluation

Congenital cardiac malformations are common developmental anomalies. In the United States, congenital heart disease is the number one cause of infant mortality from congenital malformations. Prenatal diagnosis of congenital heart defects aids treatment coordination. Our aim was to evaluate prenatal detection of serious congenital heart malformations in Clark County, Nevada.

Diagnosis of Absent Ductus Venosus in a Population Referred for Fetal Echocardiography Association With a Persistent Portosystemic Shunt Requiring Postnatal Device Occlusion

The purpose of this series was to assess the incidence, anatomic variants, and implications of an absent ductus venosus (ADV) in patients referred for fetal echocardiography.

Effectiveness and Safety of Balloon Dilation of Native Aortic Coarctation in Premature Neonates Weighing ≤2,500 Grams

Six neonates weighing < or = 2,500 g with native coarctation of the aorta underwent balloon dilation. Of the 6 neonates, 4 were female and 2 were male, with a mean age of 14 days (range 9 to 20) and a mean weight of 1,900 g (range 790 to 2,500). The procedure was acutely successful in all 6 patients; the peak gradient decreased from 38 + or - 19 mm Hg to 11 + or - 3 mm Hg. The diameter increased from 1.5 + or - 0.6 mm to 3.6 + or - 0.7 mm. Of the 6 patients, 3 had required no additional intervention at a mean of 42 months after the initial dilation, and 3 had developed restenosis a mean of 2.4 months after the initial dilation and underwent successful redilation. Of the latter 3 patients, 2 developed restenosis and underwent surgical repair 37 and 68 days after the second dilation, and 1 of these patients developed recoarctation after surgery that was treated successfully with balloon dilation 54 days after the end-to-end repair. In conclusion, premature neonates weighing < or = 2,500 g with coarctation of the aorta appear to respond acutely to balloon dilation. Some patients will have a successful long-term result after a single balloon dilation procedure. However, restenosis is common and tends to develop rapidly.

Dextrocardia: practical clinical points and comments on terminology.

Dextrocardia is defined by the authors as a right-sided heart with a base–apex axis directed rightward, resulting from a variation in cardiac development, and not used as a general term indicating any heart in the right chest. Dextrocardia occurs in approximately 0.01% of live births and can be discovered in various clinical settings and at various patient ages. The authors review their experience with dextrocardia, discuss useful clinical points that aid in evaluating complex anatomy, recount the history of dextrocardia terminology, and note the current inconsistent nomenclature.

Aorta Larger Than Pulmonary Artery in the Fetal 3-Vessel View

Objective. Fetal great artery asymmetry may accompany congenital heart disease. The fetal echocardiographic 3‐vessel view (3VV) allows assessment of the superior vena cava, ascending aorta (AA), and main pulmonary artery (MPA). Our aim was to determine the association of congenital heart disease in fetuses with an AA/MPA ratio of greater than 1. Methods. We electronically searched our fetal echocardiographic database for studies performed between March 2002 and January 2008 that showed 3VVs with AA/MPA ratios of greater than 1 and correlated the findings with the presence of congenital heart disease. Results. In 2797 fetal echocardiograms, we identified 31 fetuses with normal 4‐chamber views showing AA/MPA ratios of greater than 1 in the 3VV. Of 31 fetuses, 25 (81%) had tetralogy of Fallot (ToF) or a ToF variant, and 6 (19%) had an aortic valve abnormality or isolated dilatation of the AA. Conclusions. Screening obstetric fetal sonography showing a 3VV AA/MPA ratio of greater than 1 suggests congenital heart disease and indicates the need for comprehensive fetal echocardiography.

Serum HCG measured in the peri-implantation period predicts IVF cycle outcomes

The current study assessed the relationship between serum concentrations of human chorionic gonadotrophin (HCG) measured in the peri-implantation period and various outcome measures following blastocyst transfer in IVF cycles. The study group included 767 autologous IVF cycles, each with the transfer of two fresh blastocysts in a 6-year study period, ending 31 December 2009. Outcome measures were ectopic pregnancy, biochemical pregnancy loss, ongoing pregnancy, spontaneous abortion and multiple pregnancy. Peri-implantation serum HCG concentration measured 5 days after blastocyst transfer was highly predictive of these outcome measures. These findings suggest embryonic implantation and developmental fate are largely determined by 5 days after blastocyst transfer and that very early serum HCG measurements may be useful markers of IVF outcome.

Argatroban Anticoagulation for Cardiac Surgery with Cardiopulmonary Bypass in an Infant with Double Outlet Right Ventricle and a History of Heparin-induced Thrombocytopenia

Heparin-induced thrombocytopenia (HIT) is a life-threatening complication of heparin therapy. Anticoagulation in pediatric patients with HIT is challenging as there are no approved heparin substitutes. We report the use of the anticoagulant argatroban for cardiac surgery with cardiopulmonary bypass in an infant with double outlet right ventricle and a history of HIT.

MELD-XI Scores Correlate with Post-Fontan Hepatic Biopsy Fibrosis Scores

We tested the hypothesis that MELD-XI values correlated with hepatic total fibrosis scores obtained in 70 predominately stable, post-Fontan patients that underwent elective cardiac catheterization. We found a statistically significant correlation between MELD-XI values and total fibrosis scores (p = 0.003). Thus, serial MELD-XI values may be an additional useful clinical parameter for follow-up care in post-Fontan patients.

Enlarged Left Vitelline Vein Remnant as a Cause of Cyanosis after the Fontan Procedure: Resolution with an Amplatzer Vascular Plug

A 6-year-old girl with heterotaxy and a functional single ventricle had persistent cyanosis 4 years after a fenestrated Fontan procedure. Cardiac catheterization revealed a large venous fistula from a left-sided hepatic vein to the coronary sinus, resulting in desaturation. The anomalous vein was occluded with an Amplatzer vascular plug.

Hair-Grooming Syncope in Children

Objectives. This report describes children undergoing pediatric cardiology evaluation for syncope in which a hair-grooming trigger was determined as the stimulus. Methods. A review of our database revealed 1525 patients with syncope seen by our program of whom 111 had a hair-grooming trigger determined as a cause. Results. Of the 111 patients, 78% were girls. We found characteristic difference between boys and girls with boys experiencing syncope more during hair cutting whereas girls experienced syncope more during hair combing and brushing. Electrocardiograms and echocardiograms were performed as part of syncope evaluation and no significant abnormalities were found in either test in this patient group. Conclusions. This is the largest reported group of children presenting with syncope that had a hair-grooming trigger. Our data also include the first series of boys with the condition. The hair-grooming trigger appears to stimulate a benign form of neurocardiogenic reflex syncope.