A. Rothman

Improved prenatal detection of congenital heart disease in an integrated health care system.

The reported prenatal detection rates (PDRs) for significant congenital heart disease (sCHD) have been suboptimal, even in the current era. Changes in prenatal ultrasound policy and training may lead to improved prenatal detection of sCHD. This study analyzed the results of a policy to assess fetal cardiac outflow tracts shown by screening prenatal ultrasound using the electronic medical record (EMR). During a 6-year period, fetuses and patients younger than 1xa0year with sCHD were identified. The EMR was used to gather detection and outcome data. As an internal control within the same health care system, the PDR of only the surgical cases was compared with that of a similar group in which documentation of the fetal cardiac outflow tracts was not standard policy. Among 25,666 births, sCHD was identified in 93 fetuses or patients, yielding an incidence of 3.6 per 1,000 births. The PDR was 74.1xa0%. Detection after birth but before discharge was 20.4xa0%, and detection after discharge was 5.4xa0%. A significant improvement in the PDR of sCHD was found when a concerted effort was made to obtain fetal cardiac outflow tract views during pregnancy screening (59.3 vs 28xa0%). Within an integrated health care system and with the use of an EMR, a PDR of 74xa0% can be obtained, and 94xa0% of sCHD can be detected before discharge. A concerted program that includes documentation of fetal cardiac outflow tracts in the pregnancy screening can result in improved PDR of sCHD.

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.

Congenital Portosystemic Shunts and AMPLATZER Vascular Plug Occlusion in Newborns

Congenital portosystemic shunts (CPSs) may cause myriad manifestations. They can be detected prenatally, in infancy, or later. They may involute, cause acute symptoms, or remain unrecognized and cause chronic disease. As CPSs can require treatment, early diagnosis allows close monitoring. In symptomatic patients, interventional catheterization embolization of CPSs can be undertaken at any age.

Moving towards universal prenatal detection of critical congenital heart disease in southern Nevada: a community-wide program.

AbstractnThis study compares the current, prenatal detection rate for critical congenital heart disease in Southern Nevada with the previously reported rate, after developing and expanding a comprehensive, community-wide fetal cardiology program. For the current-period analysis, we inquired our database and electronic health records for patients born in Clark County, Nevada, with critical congenital heart disease between May 2012 and April 2014, and we compared the results with the previous period between May 2003 and April 2006. The major components of the community-wide program include fetal congenital heart disease screening via general obstetric ultrasound studies performed in obstetrician’s offices, radiology imaging centers, or maternal–fetal medicine specialty practices; subsequent referral for comprehensive fetal echocardiography performed in maternal–fetal medicine offices under the on-site supervision by fetal cardiologists; and recurring community educational programs teaching the 5-axial plane, fetal echocardiographic screening protocol to general obstetric sonographers and instructing perinatal sonographers in advanced imaging topics. For the current period, the prenatal detection rate for critical congenital heart disease in Southern Nevada was 71 versus 36xa0% for the previous period (pxa0<xa00.001). The temporal improvement in prenatal detection of critical congenital heart disease may be related to our expanded decentralized, community-wide fetal cardiology program, and our experiences may be applicable to other metropolitan areas.

Transvenous Hepatic Biopsy in Stable Fontan Patients Undergoing Cardiac Catheterization

Liver pathology complicates Fontan palliation. Previous reports established that both hepatic sinusoidal and portal fibrosis occur in patients after Fontan procedures. Past studies predominantly included symptomatic patient cohorts. Thus, the authors of this study aimed to characterize hepatic pathology via transvenous hepatic biopsies in 21 asymptomatic patients at the time of elective cardiac catheterization. Seven of these patients (33xa0%) were accompanied by an interventional procedure. Hepatic biopsies showed evidence of either sinusoidal or portal fibrosis or both in all but one patient. The findings showed a statistically significant (pxa0=xa00.005) moderately strong positive correlation between fibrosis scores and time since Fontan surgery. Additionally, no significant correlation was found between fibrosis scores and inferior vena cava pressure, pulmonary vascular resistance, platelet counts, or serum laboratory testing of hepatic function.

Temporary Transumbilical Stenting of the Ductus Venosus in Neonates with Obstructed Infradiaphragmatic Total Anomalous Pulmonary Venous Return

Newborns with obstructed infradiaphragmatic total anomalous pulmonary venous return (TAPVR) are often clinically unstable. Temporary relief of the pulmonary venous obstruction may improve their clinical status before surgical repair. Three neonates (two premature and one full-term) with obstructed infradiaphragmatic TAPVR at the ductus venosus (DV) underwent stenting of the DV by way of an umbilical venous approach. Their clinical condition stabilized, thus allowing subsequent elective surgical repair.

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.

Vascular histomolecular analysis by sequential endoarterial biopsy in a shunt model of pulmonary hypertension

The molecular mechanisms of pulmonary arterial hypertension (PAH) remain ill-defined. The aims of this study were to obtain sequential endoarterial biopsy samples in a surgical porcine model of PAH and assess changes in histology and mRNA expression during the disease progression. Differentially expressed genes were then analyzed as potential pharmacological targets. Four Yucatan micro-pigs underwent surgical anastomosis of the left pulmonary artery to the descending aorta. Endovascular samples were obtained with a biopsy catheter at baseline (before surgery) and from the left lung 7, 60, and 180 days after surgery. RNA was isolated from biopsy samples, amplified and analyzed. Dysregulated genes were linked to drugs with potential to treat or prevent PAH. With the development of PAH in our model, we identified changes in histology and in the expression of several genes with known or investigational inhibitors and several novel genes for PAH. Gene dysregulation displayed time-related variations during disease progression. Endoarterial biopsy provides a new method of assessing pulmonary vascular histology and gene expression in PAH. This analysis could identify novel applications for existing and new PAH drugs. The detection of stage- and disease-specific variation in gene expression could lead to individualized therapies.

Percutaneous fenestration closure with problematic residual native atrial septum

A variety of techniques have been utilized to close Fontan fenestrations. Among 20 patients who underwent a cardiac catheterization for fenestration closure, 3 patients had residual native atrial septum, forming an additional intermediate chamber on the pulmonary venous side of the fenestration. Three different methods were used to close these fenestrations.