Sildenafil's Early, Late Impact on Ventricular Septal Repair: Older Children Using the Double Patch.

BACKGROUND: Delayed diagnosis in children with a ventricular septal defect (VSD) is common in low- and middle-income countries. Consequently, these children present with elevated pulmonary vascular resistance (PVR) and pulmonary arterial hypertension (PAH). The study investigators introduced the double-flap valve VSD patch closure technique (DFV) in 1996 to reduce early postoperative risk. Long-term results are presented in this report. METHODS: This was a retrospective single-institution study of patients who underwent DFV between May 1996 and July 2015. Beginning in 2005, all candidates for DFV received sildenafil preoperatively and postoperatively. Preoperative catheterization data and operative, postoperative, hospital, and follow-up data were analyzed. RESULTS: A total of 40 patients underwent the DFV procedure. Patients' demographics were comparable between the sildenafil and nonsildenafil groups. One of 39 patients (2.6%) was lost to follow-up. Early mortality was 2.5% (1 of 40), and late mortality was 2.6% (1 of 38). Sildenafil improved preoperative oxygen saturation, improved preoperative hemodynamics, and shortened postoperative ventilation time. In both groups, abnormal hemodynamic values improved with a 100% oxygen challenge. The median age at late follow-up was 26.3 years (interquartile range [25%, 75%], 20.9, 29.9 years), and the median time since operation was 19.2 years (interquartile range, 11.4, 22.7 years). Current discharge survival was 97.3%. A total of 18% of patients had severe PAH in late follow-up. Multivariate analysis revealed only a baseline PVR-to-systemic vascular resistance ratio of 0.8 or greater as a significant predictor of late severe PAH. CONCLUSIONS: Long-term follow-up demonstrated that 60% of the patients will achieve normal or nearly normal pulmonary artery pressures. Furthermore, the study demonstrated that sildenafil improves preoperative hemodynamics and postoperative management. Children with VSD, elevated PVR, and PAH should not be denied operation.

Echocardiography and coronary CT angiography imaging of variations in coronary anatomy and coronary abnormalities in athletic children: detection of coronary abnormalities that create a risk for sudden death.

In a recent publication, Maron et al. reported the causes of sudden death in athletes from data collected in the US National Registry of Sudden Death in Athletes at the Minneapolis Heart Institute Registry. It is not surprising that in this study, cardiovascular disease is reported as the most common cause of sudden death in athletes (56%). The most frequently encountered cardiac pathology was hypertrophic cardiomyopathy (36% of the population who died of cardiac disease). Coronary artery anomalies of wrong sinus origin were next in frequency (17%). Less common causes attributed to coronary pathology were Kawasaki disease, origin of the left coronary artery from the pulmonary artery, atherosclerotic coronary artery disease, and myocardial bridging of the left anterior descending artery. Echocardiographic imaging along with color and pulsed-wave Doppler has been widely employed to visualize the anatomy and function of the heart and proves to be a valuable instrument in the identification of coronary artery abnormalities. Moreover, coronary CT angiography provides additional confirmatory information. This article will discuss the scope, importance, and implications of echocardiographic and coronary CT angiography imaging of the major coronary anomalies and abnormalities in young athletes who are at risk of sudden death and who otherwise have a structurally normal heart.

Frequent ventricular premature beats in children with a structurally normal heart: a cause for reversible left ventricular dysfunction?

Ventricular premature beats (VPBs) in a structurally normal heart generally are a benign condition. Rarely, however, reversible cardiomyopathy may develop. This study aimed to evaluate the incidence of cardiomyopathy among pediatric patients in a cohort with frequent VPBs and to examine the characteristics of the ventricular ectopic beats as well as therapeutic options. This study reviewed the charts of all pediatric patients between the ages of 1 day and 18 years seen at the University of Kentucky with the diagnosis of VPBs between 2003 and 2007. Frequent VPBs were defined as an ectopy burden of 5% or more in 24 h. Electrocardiograms, Holter monitors, and echocardiograms were reviewed. The review identified 28 patients (17 boys, age 13.3 ± 5.9 years, and 11 girls, age 13 ± 5.2 years) with frequent VPBs. The echocardiograms of four patients (2 boys, 14%) showed cardiomyopathy. Cardiac function normalized in all four patients, with spontaneous resolution of the VPBs (2 patients) or with antiarrhythmic therapy (2 patients). During a follow-up period of 2.7 ± 2.3 years, 32% of the patients without cardiomyopathy showed a marked spontaneous improvement in arrhythmia burden. Most of the patients showed VPBs with a left bundle branch block (LBBB) and inferior axis morphology. The most commonly associated symptoms were chest pain (17.8%) and dizziness and syncope (21.4%). Generally, VPBs in structurally normal hearts are considered benign. Rarely, a reversible cardiomyopathy can develop, requiring therapeutic intervention.

Left ventricular geometry in children and adolescents with primary hypertension.

BACKGROUND: Children with hypertension (HTN) are at increased risk for left ventricular hypertrophy (LVH). Increased left ventricular (LV) mass (LVM) by the process of remodeling in response to volume or pressure loading may be eccentric (increased LV diameter) or concentric (increased wall thickness). Our objective was to classify LV geometry among children with primary HTN and examine differences in ambulatory blood pressure (ABP). METHODS: Subjects aged 7-18 years with suspected HTN were enrolled in this cross-sectional study. ABP and LVM index (LVMI) were measured within the same 24-h period. LV geometry was classified as normal, concentric remodeling, concentric LVH, or eccentric LVH. RESULTS: Children with LVH had significantly higher ambulatory systolic BP (SBP) and diastolic blood pressure (BP) (DBP) levels and body mass index (BMI) Z-score. Sixty-eight children had HTN based upon ABP monitoring (ABPM). Thirty-eight percent of the hypertensive subjects had LVH, with equal distribution in the concentric and eccentric groups. There were significant differences in the 24-h DBP parameters when the eccentric LVH group was compared to the normal geometry and concentric LVH groups. Relative wall thickness (RWT) was inversely associated with night time DBP parameters. These relationships persisted after controlling for BMI Z-score. CONCLUSIONS: Although the risk for LVH is associated with increased SBP and BMI Z-score, those with eccentric LVH had significantly higher DBP.

Blood aldosterone-to-renin ratio, ambulatory blood pressure, and left ventricular mass in children.

OBJECTIVE: To assess the blood aldosterone-to-renin ratio (ARR) and its relationship to ambulatory blood pressure (ABP) and left ventricular mass (LVM) in children. STUDY DESIGN: A cross-sectional clinical study was conducted in 102 children (71.6% African American; 62.7% male) ranging in age from 7 to 18 years (mean, 13.6 years; median, 14 years). ABP (24-hour monitoring) was expressed as blood pressure index (BPI; mean blood pressure/95th percentile by sex and height). LVM was measured by echocardiography and expressed as an index (LVMI = g/height [m](2.7)). Regression analyses were used to estimate associations. RESULTS: African-American children had significantly lower serum aldosterone concentration and plasma renin activity compared with European-American children (aldosterone: 5.9 ng/dL vs 11.4 ng/dL, P < .0001; renin: 1.6 ng/mL/hour vs 2.8 ng/mL/hour, P = .01). However, ARR was not significantly different by race. ARR was not associated with 24-hour ABP but was significantly associated with LVMI (beta = 0.4 g/m(2.7); P = .02) after adjustment for the ratio of 24-hour urine Na to creatinine excretion, body mass index z- score, and ABP index. CONCLUSIONS: The data indicated a significant association between ARR and LVMI, but not ABP, in children, suggesting early cardiac remodeling associated with a high ARR.

Ambulatory blood pressure and increased left ventricular mass in children at risk for hypertension.

OBJECTIVE: To relate ambulatory blood pressure (ABP) to cardiac target organ measurement in children at risk for primary hypertension (HTN). STUDY DESIGN: Left ventricular mass index (LVMI) and ABP were measured concomitantly in children (6 to 18 years) at risk for hypertension using a cross-sectional study design. RESULTS: LVMI showed a significant positive correlation with 24-hour systolic blood pressure (SBP) load, SBP index (SBPI), and standard deviation score (SDS). When subjects were stratified by LVMI percentile, there were significant differences in SBP load, 24-hour SBPI, and 24-hour SSDS. The odds ratio (OR) of having elevated LVMI increased by 54% for each incremental increase of SDS in 24-hour SSDS after controlling for race and BMI (OR = 1.54, unit = 1 SDS, CI = 1.1, 2.15, P = .011) and increased by 88% for each increase of 0.1 in BPI (OR = 1.88, CI = 1.03, 3.45, P = .04). Subjects with stage 3 HTN had significantly greater mean LVMI compared with normal subjects (P = .002 by ANOVA; LMVI, 31.6 +/- 7.9 versus 39.5 +/- 10.4). CONCLUSIONS: As systolic ABP variables increase, there is greater likelihood for increased LVMI. Staging based on ABPM allows assessment of cardiovascular risk in children with primary hypertension.

Postoperative fluid balance influences the need for antihypertensive therapy following coarctation repair.

OBJECTIVE: The purpose of the investigation was to determine the effect of fluid management on the need for perioperative antihypertensive therapy following coarctation repair. DESIGN: Retrospective case study. SETTING: Pediatric intensive care unit. PATIENTS: Infants and children with repaired coarctation. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Hypertension was defined as a systolic blood pressure exceeding the 95th percentile for age. Echocardiographic variables included pre- and postoperative coarctation gradients, shortening fraction, left ventricular wall stress, and velocity of circumferential shortening. Clinical variables included age, fluid input, urine output (0-72 hrs), estimated creatinine clearance, intensive care unit stay, and diuretic use. Twenty-four patients were identified and divided into two groups. Group 1 consisted of six normotensive patients (19%, 1 wk to 2 yrs) and group 2 included 18 patients (75%, 3 wks to 12 yrs) with hypertension who required antihypertensive therapy. Group 2 patients were older (37 months vs. 3 months), received more intraoperative fluid, had lower urine output with fewer patients receiving diuretics, had diuretic therapy started later, and had longer intensive care unit stays (p < .05). When compared with group 2, 83% of group 2 patients had a net positive fluid balance between 36 and 72 hrs postoperatively. There were no differences in mean pre-/postoperative coarctation gradients, systolic function, postoperative fluids, estimated creatinine clearance, or aortic cross-clamp time. Using logistic regression analysis, we found that variables independently associated with the need for antihypertensive therapy included intraoperative fluid volume, 48- to 72-hr urine output, a positive fluid balance, and the use and timing diuretic therapy (p < .05). Subgroup analysis of infants <1 yr of age revealed similar findings. CONCLUSION: A net positive fluid balance caused by either the volume of intraoperative crystalloid infusion or a lower urine output contributes to the development of paradoxic hypertension following coarctation repair regardless of patient age. Limiting intraoperative fluids and early diuretic use may limit the need for antihypertensive therapy and shorten the intensive care unit stay.