Atrioventricular Valve Repair in Single Ventricle Physiology: Timing Matters.

OBJECTIVES: Atrioventricular valve (AVV) regurgitation in patients with single ventricle (SV) physiology severely impacts prognosis; the appropriate timing for surgical treatment is unknown. We sought to study the results of surgical treatment of AVV regurgitation in SV patients and evaluate risk factors for mortality. METHODS: Medical records of 81 consecutive patients with moderate or severe AAV regurgitation who were submitted to AVV repair or replacement during any stage of univentricular palliation between January 2013 and May 2017 were examined. We studied demographic data and perioperative factors looking for predictors that might have influenced the results. Binary logistic regression was used to assess the impact on postoperative ventricular dysfunction and mortality. RESULTS: Median age and weight were seven months (interquartile range [IQR]: 3-24) and 5.2 kg (IQR: 3.7-11.2), respectively. Seventy (86.4%) patients underwent AVV repair, and 11 (13.6%) patients underwent AVV replacement. There was an association between AVV repair effectiveness and timing of intervention (P = .004). Atrioventricular valve intervention at the time of initial surgical palliation was associated with more ineffective repairs (P = .001), while AVV replacement was more common between Glenn and Fontan procedures (P = .004). Overall 30-day mortality was 30.5% (25 patients). In-hospital mortality was 49.4%, and it was higher when AVV repair was performed concomitant with initial (stage 1) palliation (64.1% vs 35.7%; P = .01) and when an effective repair was not achieved (75% vs 41%; P = .008). Multivariable analysis identified timing concomitant with stage 1 palliation as an independent risk factor for mortality (P = .01); meanwhile, an effective repair was a protective factor against in-hospital mortality (P = .05). CONCLUSION: Univentricular physiology with AVV regurgitation is a high-risk group of patients. Surgery for AVV regurgitation at stage 1 palliation was associated with less effective repair and higher mortality in this initial experience. On the other hand, effective repair determined better outcomes, highlighting the importance of experience and the learning curve in the management of such patients.

Postoperative heart failure after stage 1 palliative surgery for single ventricle cardiac disease.

Outcomes for patients with single ventricle congenital heart disease (SV-CHD) continue to improve over time. However, the prognosis for patients who develop heart failure immediately after surgery is poorly understood. We conducted a single-center, retrospective cohort study of patients with SV-CHD, who suffered postoperative heart failure. Of 1038 cardiac surgeries performed on 621 SV-CHD patients between 2004 and 2010, 125 patients met inclusion criteria, including non-septatable anatomy, stage 1 surgery, and verified low cardiac output or heart failure state per STS definition. Overall survival was 73.2% at 2 months, 64.9% at 1 year, 60.5% at 2 years, and 54.6% at 4 years. Inotrope dependence beyond 7 days post-op yielded 45% 2-year survival versus 68% for those who weaned from inotropes within 7 days (p = 0.02). Atrioventricular valve regurgitation (AVVR) influenced survival, and patients who developed renal failure or required ECMO fared poorly, even when they survived their hospitalization. Patients with postoperative heart failure and low cardiac output syndrome constitute a high-risk population beyond the term of the initial hospitalization and have an overall mid-term survival of 55% at 4 years. Wean from inotropic therapy is not completely reassuring in this population, as they have ongoing elevated risk of cardiac failure and death in the medium term. Ventricular dysfunction, AVVR, renal failure, and need for ECMO are all important prognostic factors for mid-term mortality. Inotrope dependence for > 7 days has important implications reaching beyond the hospitalization.

Incidence and Outcomes of Patients with Functionally Univentricular Heart Born in Latvia, 2007 to 2015.

Background and Objectives: A functionally univentricular heart is the term used to describe congenital heart defects where it is impossible to restore two pumping chambers. These lesions are associated with high mortality, morbidity, and medical resource utilization. The aim of this study was to review incidence and outcomes of patients with a functionally univentricular heart at the only pediatric cardiac surgery center in Latvia. Methods: We performed a retrospective review of medical records of (i) all children with a functionally univentricular heart treated at the Clinic of Pediatric Cardiology and Cardiac Surgery, and (ii) all prenatally diagnosed cases of univentricular heart at Children's Clinical University Hospital in Latvia. We reviewed data regarding children born from January 1, 2007, to December 31, 2015. The children's cardiac anatomy and interventions were categorized in accordance with the International Pediatric and Congenital Cardiac Code (v3.3). Results: During the study period, 49 patients with a functionally univentricular heart were admitted to Children's Clinical University Hospital with a corrected incidence of 0.69 per 1000 live births per year. There were 26 patients that had a hypoplastic left ventricle, and 22 patients that had a hypoplastic right ventricle, while one patient had an indeterminate ventricle. Thirty (61.2%) patients had died by the end of data collection. Twenty-one of the 30 deaths occurred before or immediately after stage I surgical palliation. Cumulative neonatal and 5-year survival of patients with a hypoplastic right ventricle was 81.8% and 63.6%, respectively; for patients with hypoplastic left ventricle-46.2% and 17.3%, respectively. Discussion: This is the first mid-term outcome study of patients with a univentricular heart in Latvia. The high mortality reflects the challenges of a small-volume, developing congenital cardiac surgery center. Data from this study will be used as a baseline for quality improvement.

Fetal myocardial deformation in maternal diabetes mellitus and obesity.

OBJECTIVE: Experimental evidence suggests that changes in the fetal myocardium result from intrauterine effects of maternal diabetes mellitus and obesity. The aim of this study was to assess fetal cardiac function using two-dimensional speckle-tracking echocardiography to determine the effects of maternal diabetes and obesity on the fetal myocardium. METHODS: Comparative cross-sectional evaluation of myocardial function in fetuses of mothers with diabetes mellitus (FDM) or obesity (FO) and normal gestational age-matched control fetuses (FC) was performed using two-dimensional speckle-tracking echocardiography at two centers. RESULTS: In total, 178 fetuses (82 FDM, 26 FO and 70 FC) met the enrolment criteria. Mean gestational age at assessment was similar among groups: 25.3 ± 5.1 weeks for FDM, 25.0 ± 4.6 weeks for FO and 25.1 ± 4.9 weeks for FC. Mean maternal body mass index was significantly higher in FDM and FO groups compared with the FC group. Statistically significant differences in fetal cardiac function were detected between FDM and FC for global longitudinal strain (mean ± SD, -21.4 ± 6.5% vs -27.0 ± 5.2%; P < 0.001), global circumferential strain (mean ± SD, -22.6 ± 6.5% vs -26.2 ± 6.8%; P = 0.002), average longitudinal systolic strain rate (median, -1.4 (interquartile range (IQR), -1.7 to -1.1)/s vs -1.6 (IQR, -2.0 to -1.4)/s; P = 0.001) and average circumferential systolic strain rate (median, -1.4 (IQR, -1.9 to -1.1)/s vs -1.6 (IQR, -2.1 to -1.3)/s; P = 0.006). Cases of non-obese FDM also had abnormal strain parameters compared with FC. Global longitudinal strain (mean ± SD, -21.1 ± 7.5%) and average circumferential systolic strain rate (median, -1.3 (IQR, -1.8 to -1.1)/s) were significantly lower in FO compared with FC. CONCLUSIONS: Unfavorable changes occur in the fetal myocardium in response to both maternal diabetes mellitus and obesity. The long-term prognostic implications of these changes require further study. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.

Noncompaction of ventricular myocardium: a study of twelve patients.

We report 12 patients with ventricular noncompaction who were echocardiographically identified at our institution since 1991. The mean age at presentation was 3.5 years. Five patients had isolated noncompaction. Three of them had subnormal left ventricular systolic function at presentation. Noncompaction was associated with complex congenital heart defect in 3 patients. Four patients had simple congenital heart defects: pulmonary stenosis, coarctation of aorta with aberrant origin of right subclavian artery, ventricular septal defect, and partial anomalous pulmonary venous return. The observed rhythm abnormalities were Wolff-Parkinson-White syndrome and paroxysmal supraventricular tachycardia, bigemini ventricular extrasystoles, and left bundle branch block. A transvenous pacemaker was implanted in a patient because of complete heart block. Noncompaction of the ventricular myocardium is rare. Our patients clearly represent the clinical and morphological spectrum of this disorder. Distinct morphological features can be diagnosed on 2-dimensional echocardiography.