The Early "Unnatural" History Following Surgical Repair of Ventricular Septal Defects.

INTRODUCTION: Surgical outcomes for simple ventricular septal defects (VSD) have been excellent in the past three decades. For this project, the timing of resolution of left-sided dilation and mitral regurgitation (MR) following VSD repair was assessed. METHODS: Echocardiographic data surrounding surgery of 42 consecutive children who underwent surgical patch repair of a VSD were reviewed. The echocardiograms were reviewed up to a mean of 12 months post-operatively (range 9 - 14 months). Quantitative data indexed to body surface area including left atrial (LA) volume, mitral valve annulus diameter, and left ventricular end-diastolic dimension (LVEDD) was analyzed. RESULTS: The majority of our pre-surgical cohort had only trace (44%) or no MR (31%), with a small proportion having mild (16%) or moderate MR (9%). No patients had moderate or greater MR following repair at follow-up. The median mitral valve annular Z-score was 1.8 (SD 1.6; range: -1.2 to 4.1) pre-operatively, improving to a 0.6 (range: -1.7 to 2.4; p < 0.001) at follow-up. LA dilation was present in 70% of patients, with a median LA volume Z-score of 1.1 (range: -2.6 to 15.5), decreasing to 13% median Z-score -1.2 (range: -3.5 to 2.9; p < 0.001) at follow-up. LV dilation was present in 81% of pre-operative patients with a median LVEDD Z-score of 3.0 (range: -2.0 to 7.9). There was significant improvement in qualitative assessment of LV enlargement (25%) with a median LVEDD Z-score of 0.5 (range: -2.1 to 2.9; p < 0.001) at follow-up. Discharge echocardiogram was performed at a mean of 5.7 days (range: 3 - 12 days) following surgery. CONCLUSIONS: Normalization of LA, mitral valve annulus, and LV size occurred within the first three months in the majority of patients, with significant changes occurring within the first post-operative week following surgical repair for VSD.

Effect of Intravenous Phenobarbital on Left Ventricular Myocardial Contractility Determined by Echocardiography in Children.

INTRODUCTION: Animal studies and rare human studies have suggested a negative effect of barbiturates on cardiac function. Although intravenous (IV) phenobarbital is used routinely in children in the clinical setting, studies in children are lacking. We performed a study to evaluate effect of IV phenobarbital loading on myocardial systolic function of children. METHODS: In a prospective pilot study in children without congenital heart defects, the effect of IV phenobarbital was evaluated on the left ventricular systolic function measured by ejection fraction (EF) by Simpson's method via an echocardiogram. Any child less than 18 years of age who received IV loading dose of at least 20 mg/kg of phenobarbital given as an infusion over 20 - 30 minutes for various medical indications was eligible to take part in the study. Three measurements of EF by an echocardiogram were made: before loading dose, 30 minutes after completion of the loading dose, and prior to the first maintenance dose. Relevant clinical data were recorded, including vital signs, immediately prior to each echocardiogram. Change of function as measured by EF over time was analyzed using linear mixed modeling methods. For this study, significant change in blood pressure was defined as a drop of at least 20 mmHg in systolic blood pressure. RESULTS: Ten children (70% female, age range two days to 8.2 years) were enrolled. Three had hypotension with a drop of systolic blood pressure greater than 20 mmHg from baseline. On examining the trajectory of EF on each individual graphically, the left ventricular EF tended to fall immediately following phenobarbital therapy and return to baseline on re-evaluation. These trajectories were statistically significant for EF. CONCLUSIONS: Phenobarbital had a direct and transient depressant effect on systolic function of the myocardium in one third of the cases. The depression in EF appeared to be transient with return to baseline in less than 24 hours. We recommend close monitoring with anticipation of decreased function in children when using IV phenobarbital.

Anomalous aortic origin of the right coronary artery from the non-coronary sinus of Valsalva.

We report a rare case of anomalous aortic origin of the right coronary artery from the posterior/non-coronary sinus of Valsalva in a 9-year-old male diagnosed during the workup of premature ventricular contractions. The finding was initially noted on transthoracic echocardiogram and further confirmed with computed tomography. The anomalous coronary artery shows a wide ostium with no intramural or interarterial course.

Innocent Heart Murmur.

Heart murmur is the most common reason for a referral to a pediatric cardiologist. Virtually all children have a heart murmur during their childhood. Less than 1% of murmurs are pathological in children. Innocent/functional heart murmur is the most common type of heart murmur. There are multiple theories proposed to identify etiology of innocent heart murmur with varying consensus, but everybody agrees that innocent heart murmur does not carry any morbidity or mortality risk. Even today, heart murmur is associated with high physician uncertainty and parental anxiety. Extensive cardiac evaluation for such a benign finding is also associated with high health care utilization and cost. This article attempts to review this long-known finding which continues to remain a diagnostic challenge.

Coarctation of Aorta in Children.

Coarctation of aorta (CoA) is a discrete narrowing in aorta causing obstruction to the flow of blood. It accounts for 6-8% of all congenital heart diseases. With advances in fetal echocardiography rate of prenatal diagnosis of coarctation of aorta has improved but it still remains a challenging diagnosis to make prenatally. Transthoracic echocardiography is mainstay of making initial diagnosis and routine follow-up. Cardiac magnetic resonance imaging (MRI) and computed tomography (CT) are great advanced imaging tools for two-dimensional and three-dimensional imaging of aortic arch in complex cases. Based on type of coarctation, size of patient, severity of lesion, and associated abnormalities various management options like surgical treatment, transcatheter balloon angioplasty and transcatheter stent implantation are available. There is significant improvement in long-term survival from pre-surgical era to post-surgical era. But, among the postsurgical era patients, the long-term survival has not significantly changed between older and contemporary cohort. Patients with coarctation of aorta need lifelong follow-up event after successful initial intervention.

Myocardial Ischemia with Cannabinoid Use in an Adolescent.

A 16-year-old male presented to the emergency department with chest pain after smoking a synthetic cannabinoid from a vape pen. He had rising troponin I levels, and his exercise stress echocardiogram showed distal apical and septal hypokinesis that resolved at six-month follow-up. This case report raises concern about cardiac ischemia related to synthetic cannabinoid abuse in the pediatric population in the current era of cannabis legalization.

Development of aortic coarctation following device closure of patent ductus arteriosus.

A patient who had transcatheter closure of a large patent ductus arteriosus in early infancy developed aortic coarctation during follow-up. Initially, balloon angioplasty and subsequent stent implantation successfully relieved the aortic obstruction. Avoidance of use of large devices in small babies and modification of current devices so that they do not cause aortic obstruction may result in better outcomes. Once aortic obstruction develops, transcatheter management is useful in relieving the obstruction.

Increased left main coronary artery dimensions in children with sickle cell disease.

For adults with sickle cell disease (SCD), cardiac changes are well described and may account for up to one-fourth of disease-related mortality. Much less is known about heart disease in pediatric SCD patients. This retrospective study analyzed a contemporary cohort of children with SCD followed at a single tertiary care center. The study aimed to evaluate the effect of SCD on left main coronary artery (LMCA) dimensions in the contemporary pediatric population. The echocardiography database was searched for studies of children with SCD from 2000 to 2009. The LMCA diameter was remeasured offline from digital images. Digital echocardiographic studies of 68 children (35 boys) 11.7 ± 4.6 years of age with SCD were analyzed. All the patients had normal systolic function as measured by fractional shortening. The left ventricular end-diastolic dimension (LVEDD) for 34 % of the pediatric SCD patients showed a dilated LV. In 24 % of the children, a LV mass index (LVMI) greater than 50 g/m(2.7) denoted LV hypertrophy. Application of sex-specific normal values showed that 60 % of the boys and 33 % of the girls had an increased LVMI. The LMCA diameter was enlarged in 43 % of the SCD children, including 26 % with an LMCA z-score higher than 3. This study found a higher incidence of LMCA dilation in a pediatric referral population with SCD. The findings also validated an increased LVMI in the contemporary SCD patient population, which was noted previously. This study adds the need to include assessment of coronary arteries in cardiac evaluation of SCD patients, and special attention should be paid to patients with a high-normal to high coronary z-score.