Left heart hypoplasia and neonatal aortic arch obstruction: Is the rhodes left ventricular adequacy score applicable? (original) (raw)
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European Journal of Cardio-Thoracic Surgery, 2009
A newborn presented with severe aortic valve stenosis and a borderline hypoplastic left ventricle due to disproportionate left ventricular hypertrophy (maternal diabetes). The aortic valve was balloon dilated and the infant tolerated a biventricular circulation. However, severe retrograde pulmonary hypertension and mitral regurgitation developed, indicating that biventricular circulation was not possible at that stage. A hybrid approach with ductal stenting, atrial septostomy and bilateral dilatable pulmonary artery band placement was followed on day 25. This allowed the left ventricle several months to adapt to lower pressure and normoglycemic conditions. At re-evaluation after 8 months biventricular repair appeared possible: the ductus was closed with Amplatzer occluders and the pulmonary artery bands were opened up with bilateral balloon angioplasty of the dilatable bands. At the age of 3 years, the infant is doing well with a biventricular circulation and normal pulmonary artery pressure. The hybrid approach allowed adequate time (months) for careful consideration and acted as a bridge to biventricular repair in this infant. #
Journal of Cardiothoracic Surgery, 2015
Background: Leaving an inter-atrial communication (IAC) open for left atrial decompression is often recommended in neonates with aortic arch obstruction undergoing primary repair. In this study, outcomes in these patients were compared to those with intact atrial septum after repair. Methods: Between 2000 and 2013, 53 consecutive neonates with severe aortic arch obstruction (hypoplasia: n = 45, interruption: n = 8) underwent primary repair from an anterior approach. Median age and weight were 8 days (range: 2-30) and 3.2 kg (range: 2.4-4.4), respectively. Cardiac morphology included a ventricular septal defect (VSD, large: n = 28, small: n = 7), malposition of great arteries (n = 10), and severe left ventricular outflow tract obstruction (LVOTO, n = 10). During corrective surgery IAC was closed (group-I, n = 37) or partially left-open (group-II, n = 16). Primary endpoints were hospital death, and re-intervention (surgery and/or balloon) due to aortic arch re-coarctation or recurrent LVOTO. Statistically significant variables by univariate analysis were incorporated in the corresponding multivariable regression model. Results: Regarding morphological discrepancies more patients in group-II presented with LVOTO (p = 0.05), or the combination of arch hypoplasia, intact ventricular septum and normal ventriculo-arterial connection (p = 0.017). Hospital mortality was 8.1% in group-I and 37.5% in group-II (p = 0.016). Re-intervention was performed in 13 patients (group-I: n = 6 vs. group-II: n = 7) due to aortic arch re-coarctation (n = 12) and/or recurrent LVOTO (n = 3), and resulted in a Kaplan-Meier freedom from re-intervention of 87 ± 6% and 79 ± 8% in group-I, and 64 ± 14% and 64 ± 14% in group-II after 1 and 5 years, respectively (p = 0.016). Multivariate analysis revealed LVOTO as an independent risk factor for hospital death (p = 0.042), whereas both LVOTO and left-open IAC (p = 0.001 and 0.01) were independent risk factors for re-intervention.
Staged Biventricular Repair After Hybrid Procedure in High-Risk Neonates and Infants
World Journal for Pediatric and Congenital Heart Surgery, 2019
Background: Single-stage biventricular repair remains a challenging and difficult decision in high-risk newborns and early infants with the presence of left ventricular outflow tract obstruction (LVOTO) or borderline hypoplasia of the left ventricle (LV). Methods: Six high-risk patients underwent the initial hybrid procedure (bilateral pulmonary banding + ductal stenting) for staged biventricular repair. Their median age was 17 days (range: 7-55 days). The diagnosis was interrupted aortic arch (IAA), ventricular septal defect (VSD), and LVOTO (n = 3); IAA and VSD (n = 1); and aortic annular hypoplasia, aortic arch hypoplasia, VSD, and LVOTO (n = 1). The last patient had borderline LV with large atrial septal defect (ASD) and aortic arch hypoplasia. The patient with borderline LV had also ASD closure with small fenestration. Results: One patient died of sepsis after the hybrid procedure. Other patients underwent biventricular repair 8 to 13 months later. Three patients had convention...
Short‐term results in infants with multiple left heart obstructive lesions
Congenital Heart Disease, 2019
Objective: Deciding on a surgical pathway for neonates with ≥2 left heart obstructive lesions is complex. Predictors of the successful biventricular (2V) repair in these patients are poorly defined. The goal of our study was to identify patients who underwent the 2V repair and assess anatomic and echocardiographic predictors of success. Design: Infants born between July 2015 and August 2017 with ≥2 left heart obstructive lesions with no prior interventions were identified (n = 19). Patients with aortic or mitral valve (MV) atresia and critical aortic stenosis were excluded. Initial echocardiograms were reviewed for aortic, MV, tricuspid valve annulus size, and left (LV) and right (RV) ventricle diastolic longitudinal dimensions. The valve morphology and presence of a ventricular septal defect (VSD) and coarctation were assessed. Clinical outcomes included successful 2V repair, complications, and repeat interventions or surgeries. Failed 2V repair was defined as a takedown to single ventricle (1V) physiology, cardiac transplantation, or death. Results: For 2V repair, 14/19 patients were selected and for 1V, 5/19 patients were selected. Initial surgical procedures of the 2V group were simple coarctation repair (5), complex coarctation/arch reconstruction +/− septal defect closure (6), hybrid stage 1 (2), and none (1). Three of the 2V patients required reintervention in the first 90 days. The LV to RV diastolic longitudinal ratio >0.75 and mitral/tricuspid ratio of <0.8 were observed in 13/14 of the 2V patients. The LV:RV ratio and the aortic valve z score were significantly larger in the 2V group compared to the 1V group. All patients in the 1V group had a nonapex forming LV. There was no mortality with follow-up to three years of age. Conclusions: This study showed excellent short-term and midterm surgical results in the 2V population. The LV:RV diastolic longitudinal ratio may be a useful tool in the risk stratification of a successful 2V repair even in cases with a small MV.
The Journal of Thoracic and Cardiovascular Surgery, 2005
We sought to determine the prevalence of outcomes and associated patient and management factors for neonates with interrupted aortic arch. Methods: From 1987 to 1997, a total of 472 neonates were enrolled prospectively from 33 institutions. Competing risks methodology was used to determine simultaneous risk and associated incremental risk factors for death, initial and subsequent left ventricular outflow tract procedures, and arch reinterventions. Results: Overall survival was 59% at 16 years after study entry but improved with successive birth cohort. In general, risk factors for death in each of the competing risks analyses included lower birth weight, younger age at study entry, type B interrupted aortic arch, and major associated cardiac anomalies. Of 453 patients who had interrupted aortic arch repair, after 16 years 33% had died and 28% had undergone an arch reintervention. Reintervention was more likely for those who had truncus arteriosus repair, interrupted aortic arch repair by a method other than direct anastomosis with patch augmentation, and the use of polytetrafluoroethylene as either an interposition graft or a patch. From study entry, competing risks after 16 years showed that 28% had died and 34% had undergone an initial left ventricular outflow tract procedure. Initial left ventricular outflow tract procedure was more likely for those with single ventricle, type B interrupted aortic arch, bicuspid aortic valve, or anomalous right subclavian artery. Among those who had undergone an initial left ventricular outflow tract procedure, after 16 years 37% had died and 28% had undergone a second procedure. Conclusion: Anatomic features affect mortality and initial left ventricular outflow tract procedures, whereas characteristics of the arch repair affect arch reintervention. I nterrupted aortic arch (IAA) occurs infrequently, and although most patients have only an isolated ventricular septal defect (VSD), more complex associated cardiovascular anomalies may occur. In addition, a spectrum of left ventricular outflow tract (LVOT) obstructive lesions, particularly subaortic stenosis, may further complicate management. Although recent reports have reflected improvements in operative survival, further interventions are often required to address residual or recurrent left heart outflow or arch repair obstructions. A previous Congenital Heart Surgeons Society (CHSS) study reported outcomes for 183 neonates entered from 1987 to 1992.
Feasibility and Safety of Biventricular Repair in Neonates with Hypoplastic Left Heart Complex
Pediatric Cardiology, 2014
Hypoplastic left heart syndrome is a spectrum of structural cardiac malformations characterized by variable underdevelopment of the left heart-aorta complex. A minority of patients having a milder degree of left ventricular hypoplasia, described as hypoplastic left heart complex (HLHC), may be selected for biventricular repair. The objective of this study was to assess the outcome of the biventricular approach in HLHC. We evaluated retrospectively 30 neonates diagnosed with HLHC from the ''12 de Octubre'' University Hospital, following established criteria. We analyzed the echocardiographic data recorded just after birth and at last follow-up after surgery. All patients were operated on in the neonatal period using various surgical techniques. There were no early deaths and only 1 late death after a mean follow-up of 62.9 ± 43.8 months. All patients presented a significant growth of the left ventricular structures, with a Z-score increase of 1.17 ± 1.05 for mitral annulus, 1.72 ± 1.23 for aortic annulus, and 1.33 ± 1.46 for left ventricular end-diastolic diameter. Postoperatively, 18 patients showed a left valvular stenosis, and 17 patients underwent a reoperation and/or an interventional procedure. Freedom from surgery or interventional catheterizations at 1, 3 and 5 years was 53, 49 and 43 %, respectively. The 29 current survivors are all in a good functional status. In our experience, we achieved good results from biventricular repair in patients with HLHC, with a significant growth of left heart structures and an excellent clinical status at a medium-term follow-up. Nevertheless, there was a high rate of reoperations and/or interventional catheterizations.