Early and long-term results of ventricular septal defect repair in children with severe pulmonary hypertension and elevated pulmonary vascular resistance by the double or traditional patch technique.

OBJECTIVES: In upper-income countries, infants undergo low-risk ventricular septal defect (VSD) repair. Children in low- and middle-income countries frequently present at older ages with elevated pulmonary vascular resistance (PVR) and pulmonary arterial hypertension (PAH). Expensive interventions for pulmonary hypertensive crisis are not available, and children are often denied an operation due to the mortality risk. We report our early and late experiences with these patients who underwent VSD closure by traditional patch (TP) or double patch (DP) techniques. METHODS: We extracted data from patients with VSD and PAH who underwent VSD closure operations from 1996 to 2016. Information regarding cardiac catheterizations, operations, time in the intensive care unit and follow-up information was found. We identified 129 children and analysed the differences between the TP (89/129) and the DP (40/129) in unidirectional groups. After 2005, the patients were pretreated with sildenafil 3 months before catheterization. RESULTS: The TP group was younger (P < 0.001). Hospital mortality (2.5%, DP; 10.1%,TP) was not significantly different (P = 0.17) between the 2 groups despite significant differences in baseline PVR, PVR/systemic vascular resistance and the number of children with a saturation less than 90%. The long-term survival and regression of PAH were better in the DP group (survival over 97% vs 93% in the TP group). Moreover, 61% of children with DP and 75% of those with TP had normal to mild elevations in pulmonary artery pressures at the last follow-up examination (P = 0.046). CONCLUSIONS: The DP operation is a low-risk procedure. Moreover, the long-term outcome with the use of this technique in high-risk patients with VSD and high PVR and PAH is favourable with respect to survival and showed a decrease in PAH at the last follow-up.

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.

Cost-effectiveness of Humanitarian Pediatric Cardiac Surgery Programs in Low- and Middle-Income Countries.

Importance: Endorsement of global humanitarian interventions is based on either proven cost-effectiveness or perceived public health benefits. The cost-effectiveness and long-term benefits of global humanitarian pediatric cardiac surgery are unknown, and funding for this intervention is insufficient. Objectives: To determine the cost-effectiveness of the intervention (multiple 2-week-long humanitarian pediatric cardiac surgery program assistance trips to various low- and middle-income countries [LMICs]) and to produce a measure of the long-lasting effects of global humanitarian programs. Design, Setting, and Participants: International, multicenter cost-effectiveness analysis of a cohort of children (aged <16 years) undergoing surgical treatment of congenital heart disease during 2015 in LMICs, including China, Macedonia, Honduras, Iran, Iraq, Libya, Nigeria, Pakistan, Russia, and Ukraine. The study also assessed estimated improvement in the United Nations Human Development Indicators (life expectancy, years of schooling, and gross national income) for each individual survivor, as a proxy for long-term benefits of the intervention. Main Outcomes and Measures: The primary outcome was cost-effectiveness of the intervention. The secondary outcomes were potential gains in life expectancy, years of schooling, and gross national income per capita for each survivor. Results: During 2015, 446 patients (192 [43%] female; mean [SD] age, 3.7 [5.4] years) were served in 10 LMICs at an overall cost of $3 210 873. Of them, 424 were children. The cost-effectiveness of the intervention was $171 per disability-adjusted life-year averted. Each survivor in the cohort (390 of 424) potentially gained 39.9 disability-adjusted life-years averted, 3.5 years of schooling, and $159 533 in gross national income per capita during his or her extended lifetime at purchasing power parity and 3% discounting. Conclusions and Relevance: Humanitarian pediatric cardiac surgery in LMICs is highly cost-effective. It also leaves behind a lasting humanitarian footprint by potentially improving individual development indices.

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.

Predominance of nocturnal hypertension in pediatric renal allograft recipients.

Hypertension is common in children with end-stage renal disease who have undergone renal transplantation. We performed ambulatory blood pressure monitoring (ABPM) in renal allograft recipients who were on stable maintenance immunosuppressive medications and were more than six months post-transplant. Echocardiographic measurement of left ventricular mass index (LVMI) was obtained at the time of ABPM. Twenty-nine children with a mean age of 14.8 yr (8-18 yr) were evaluated 4.3 yr (0.6-12.8 yr) after deceased donor (n = 13) or living donor (n = 16) transplantation. BP levels were higher during sleep compared with when awake using the 95th percentile to standardize mean BP for each period: mean BP was expressed as a standard deviation score (SDS) for each time period, awake vs. sleep: systolic (s) BP SDS were 0.43 +/- 1.3 vs. 1.29 +/- 1.2 (p < 0.001) and diastolic (d) BP SDS were 0.04 +/- 1.3 vs. 1.34 +/- 1.2 (p < 0.001). Significant differences between awake and sleep BP were also confirmed using the mean BP for each period expressed as a BPI. Hypertension (HTN) during sleep was more common than awake HTN. Based upon BPI, 21% had sHTN when awake compared with 48% during sleep and 7% had dHTN when awake compared with 41% during sleep (p < 0.05). Based upon mean BP load, 38% had sHTN when awake compared with 55% during sleep and 21% demonstrated dHTN when awake compared with 52% during sleep (p < 0.05). Left ventricular mass (LVM) was abnormally increased in six of 17 children (35%); LVM was not correlated with BP. Children prescribed angiotensin converting enzyme inhibitors or angiotensin II receptor blockers (ACEi/ARB) had significantly lower systolic BP compared with those on calcium channel blocking agents (CCB). Mean sSDS was -0.11 +/- 1.1 in those children on ACEi/ARB compared with 1.6 +/- 1.2 in those on CCB (p = 0.02): sSDS during sleep was significantly lower in the ACEi/ARB group compared with CCB (0.70 +/- 1.1 vs. 2.0 +/- 1.1, p = 0.04). Isolated nocturnal HTN is more common than daytime HTN among clinically stable pediatric renal allograft recipients. Detection and treatment of nocturnal HTN in pediatric allograft recipients could potentially affect graft survival.

[Transluminal balloon valvuloplasty in neonates and infants with critical aortic stenosis]. / Transluminalna balonska valvuloplastika u novorodencadi i dojencadi s kriticnom aortnom stenozom.

The purpose of this study was to evaluate children who underwent balloon valvuloplasty due to critical aortic stenosis following clinical (low cardiac output, cardiogenic shock, congestive heart failure) and echocardiographic criteria (morphological evidence of left ventricular hypertrophy, with depression of left ventricular function, irrespective of transvalvular gradient). We assessed the effectiveness of balloon valvuloplasty in 5 children (all male) who were submitted to aortic valve balloon dilatation over 3.5 years (10.1998-05.2003). The age at dilatation was 29+/-24 days, BW 3.92+/-0.82 kg and BSA 0.24+/-0.03 m2. In all children the balloon valvuloplasty was performed with manual inflation of balloon at 4-6 bars through the femoral artery. The mean systolic pressure gradient across the aortic valve decreased from 68+/-20.5 mmHg to 9+/-10.95 mmHg, i.e. by 85%, (p<0.01). Aortic valve ring diameter was 9.2+0.84 mm, and balloon/aortic ring ratio 0.8-0.04. The degree of aortic insufficiency immediately after the dilatation did not significantly increase. Dilatation was performed without complications. Long term results were evaluated in all patients 3.2 - 54 months after valvuloplasty and revealed the continuously increasing residual aortic valve gradient (Doppler measurement) 33+/-10.95 mmHg to be significantly lower (p<0.01) than before valvuloplasty. None of the children was showing clinical symptoms of the disease. According to echocardiographic analysis two of them developed aortic valvar insufficiency grade II, two had trivial insufficiency, one was without insufficiency. One child is an candidate for the Ross procedure in future (gradient 50 mmHg, insufficiency grade II, age 4.5 years.). Balloon valvuloplasty provides effective interventional method in the treatment of the neonates and infants with critical aortic stenosis.

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.