Priya Sekar

Prenatal Head Growth and White Matter Injury in Hypoplastic Left Heart Syndrome

Children with hypoplastic left heart syndrome (HLHS) have an increased prevalence of central nervous system (CNS) abnormalities. The extent to which this problem is due to CNS maldevelopment, prenatal ischemia, postnatal chronic cyanosis and/or multiple exposures to cardiopulmonary bypass is unknown. To better understand the etiology of CNS abnormalities in HLHS, we evaluated 68 neonates with HLHS; in 28 cases, both fetal ultrasound and echocardiogram data were available to assess head size, head growth and aortic valve anatomy (atresia or stenosis). In addition, we evaluated neuropathology in 11 electively aborted HLHS fetuses. The mean head circumference percentile in HLHS neonates was significantly smaller than HLHS fetuses (22 ± 2% versus 40 ± 4%, p < 0.001). A significant decrease in head growth, defined as a 50% reduction in head circumference percentile, was observed in half (14/28) of HLHS fetuses and nearly a quarter (6/28) were already growth restricted (≤10%) at the time of initial evaluation. Brains from HLHS fetuses demonstrated chronic diffuse white matter injury of varying severity. These patterns of prenatal head growth and brain histopathology identify a spectrum of abnormal CNS development and/or injury in HLHS fetuses.

Aortic arch recoarctation after the Norwood stage I palliation: the comparative accuracy of blood pressure cuff and echocardiographic Doppler gradients in detecting significant obstruction.

OBJECTIVE Aortic arch recoarctation is responsible for significant morbidity and mortality after the Norwood Stage I procedure. Cuff blood pressure (BP) gradients and echocardiographic Doppler gradients are routinely used as noninvasive screening tests for early detection, but accuracy has not been systematically tested. We sought to evaluate the ability of cuff BP and Doppler gradients, measured at routine outpatient clinic visits, to predict significant arch obstruction in single ventricle patients after the Norwood operation. DESIGN Consecutive patients who underwent Norwood operation at our institution were identified retrospectively. Cuff and echocardiographic gradients measured prior to the pre-Glenn catheterization were compared to peak-to-peak systolic neoaortic arch gradients obtained at catheterization. Statistical analyses, including Receiver Operator Characteristic (ROC) curves, were performed using different cutpoints for cuff and echocardiographic gradients, evaluating their ability to predict a clinically significant catheter gradient. RESULTS Data were obtained in 68 patients. Echocardiographic gradient cutpoints were more sensitive but less specific than cuff BP gradient cutpoints at detecting a catheter gradient > or = 10 mm Hg. Echo gradients > or = 20 mm Hg showed 85% sensitivity and 95% specificity in detecting a systolic catheter gradient > or = 10 mm Hg. CONCLUSION chocardiographic Doppler outperforms cuff BP as a sensitive noninvasive screening tool for early detection of significant arch obstruction in infants after the Norwood operation.

Diagnosis of congenital heart disease in an era of universal prenatal ultrasound screening in southwest Ohio

OBJECTIVES Diagnostic ultrasound is widespread in obstetric practice, yet many babies with major congenital heart disease remain undiagnosed. Factors affecting prenatal diagnosis of major congenital heart disease are not well understood. This study aims to document prenatal detection rates for major congenital heart disease in the Greater Cincinnati area, and identify factors associated with lack of prenatal diagnosis. METHODS All living infants diagnosed with major congenital heart disease by 4 months of age at our centre were prospectively identified. Prenatal care data were obtained by parent interview. Neonatal records were reviewed for postnatal data. Obstetricians were contacted for diagnostic ultrasound data. RESULTS A total of 100 infants met the inclusion criteria. In all, 95 infants were analysed, of whom 94 were offered diagnostic ultrasound. In all, 41 had a prenatal diagnosis of major congenital heart disease. The rate of prenatal detection varied by cardiac lesion, with aortic arch abnormalities, semilunar valve abnormalities, and venous anomalies going undetected in this sample. Among subjects without prenatal detection, the highest proportion consisted of those having Level 1 diagnostic ultrasound only (66%). Prenatal detection was not significantly influenced by maternal race, education level, income, or insurance type. CONCLUSIONS Despite nearly universal diagnostic ultrasound, detection rates of major congenital heart disease remain low in southwest Ohio. An educational outreach programme including outflow tract sweeps for community-level obstetrical personnel may improve detection rates.

Unilateral pulmonary edema and acute rheumatic fever

Although the diagnostic criteria for acute rheumatic fever (ARF) are well known, a high index of suspicion is necessary in order to assure timely diagnosis and appropriate treatment. We present a case of an 8-year-old child who presented with unilateral pulmonary edema secondary to acute mitral insufficiency due to ARF. ARF should be considered in the differential diagnosis of unilateral pulmonary edema in children.

Prenatal ABO/RHD Genotyping: A New Paradigm to Allow for Fresh Whole Blood for Cardiopulmonary Bypass in the Immediate Newborn Period

Compared to standard component therapy, fresh whole blood (FWB) offers potential benefits to neonates undergoing cardiopulmonary bypass (CPB) in the context of open cardiac surgery: decreased blood loss and subsequent risk of volume overload, improved coagulation status, higher platelet counts during and following CPB, circumvention of limited vascular access, and significantly reduced donor exposures. Obtaining FWB, however, entails 2–5 days of preparation, which often precludes its availability for neonates requiring CPB in the immediate newborn period. Using a multidisciplinary approach and molecular ABO/RHD genotyping on amniotic fluid, we developed a protocol to allow procurement of FWB for timed delivery followed by open cardiac surgery. Eligible subjects include patients undergoing genetic amniocentesis following the diagnosis of a fetal cardiac anomaly likely to require open surgical repair in the initial days after birth. This protocol has been successfully implemented following prenatal diagnosis of severe fetal cardiac anomalies. Taking advantage of the prenatal time period and the ability to perform fetal blood typing prenatally using molecular genotyping makes possible a new paradigm for the availability of FWB for CPB to improve perioperative, short-term, and long-term outcomes in a population comprised of some of the smallest and sickest patients who will undergo CPB.

Non-rheumatic streptococcal myocarditis mimicking acute myocardial infarction in an adolescent male

An adolescent male with a recent history of streptococcal pharyngitis presented with severe substernal chest pain, troponin leak, and ST-segment elevation, which are suggestive of acute inferolateral myocardial infarction. The coronary angiogram was normal. The patient was subsequently diagnosed with non-rheumatic streptococcal myocarditis. He was treated with amoxicillin and had excellent recovery. Non-rheumatic streptococcal myocarditis is an important mimic of acute myocardial infarction in young adults.

Red flags: a case series of clinician–family communication challenges in the context of CHD

We describe three cases of newborns with complex CHD characterised by communication challenges. These communication challenges were categorised as patient, family, or system-related red flags. Strategies for addressing these red flags were proposed, for the goal of optimising care and improving quality of life in this vulnerable population.