Skip to main content
Erschienen in: Pediatric Cardiology 6/2017

04.07.2017 | Original Article

The Infant with Aortic Arch Hypoplasia and Small Left Heart Structures: Echocardiographic Indices of Mitral and Aortic Hypoplasia Predicting Successful Biventricular Repair

verfasst von: Jennifer M. Plymale, Peter C. Frommelt, Melodee Nugent, Pippa Simpson, James S. Tweddell, Amanda J. Shillingford

Erschienen in: Pediatric Cardiology | Ausgabe 6/2017

Einloggen, um Zugang zu erhalten

Abstract

In infants with aortic arch hypoplasia and small left-sided cardiac structures, successful biventricular repair is dependent on the adequacy of the left-sided structures. Defining accurate thresholds of echocardiographic indices predictive of successful biventricular repair is paramount to achieving optimal outcomes. We sought to identify pre-operative echocardiographic indices of left heart size that predict intervention-free survival in infants with small left heart structures undergoing primary aortic arch repair to establish biventricular circulation (BVC). Infants ≤2 months undergoing aortic arch repair from 1999 to 2010 with aortic and/or mitral valve hypoplasia, (Z-score ≤−2) were included. Pre-operative and follow-up echocardiograms were reviewed. Primary outcome was successful biventricular circulation (BVC), defined as freedom from death, transplant, or single ventricular conversion at 1 year. Need for catheter based or surgical re-intervention (RI), valve annular growth, and significant late aortic or mitral valve obstruction were additional outcomes. Fifty one of 73 subjects (79%) had successful BVC and were free of RI at 1 year. Seven subjects failed BVC; four of those died. The overall 1 year survival for the cohort was 95%. Fifteen subjects underwent a RI but maintained BVC. In univariate analysis, larger transverse aorta (p = 0.006) and aortic valve (p = 0.02) predicted successful BVC without RI. In CART analysis, the combination of mitral valve (MV) to tricuspid valve (TV) ratio ≤0.66 with an aortic valve (AV) annulus Z-score ≤−3 had the greatest power to predict BVC failure (sensitivity 71%, specificity 94%). In those with successful BVC, the combination of both AV and MV Z-score ≤−2.5 increased the odds of RI (OR 3.8; CI 1.3–11.4). Follow-up of non-RI subjects revealed improvement in AV and MV Z-score (median AV annulus changed over time from −2.34 to 0.04 (p < 0.001) and MV changed from −2.88 to −1.41 (p < 0.001), but residual mitral valve stenosis and aortic arch obstruction were present in one-third of subjects. In this cohort of infants requiring initial aortic arch repair with concomitant small left heart structures, successful BVC can be predicted from combined echocardiographic indices. In this complex population, 1 year survival is high, but the need for RI and the presence of residual lesions are common.
Literatur
1.
Zurück zum Zitat Hickey EJ et al (2007) Critical left ventricular outflow tract obstruction: the disproportionate impact of biventricular repair in borderline cases. J Thorac Cardiovasc Surg 134(6):1429–1436 (discussion 1436–7) CrossRefPubMed Hickey EJ et al (2007) Critical left ventricular outflow tract obstruction: the disproportionate impact of biventricular repair in borderline cases. J Thorac Cardiovasc Surg 134(6):1429–1436 (discussion 1436–7) CrossRefPubMed
2.
Zurück zum Zitat Rhodes LA et al (1991) Predictors of survival in neonates with critical aortic stenosis. Circulation 84(6):2325–2335CrossRefPubMed Rhodes LA et al (1991) Predictors of survival in neonates with critical aortic stenosis. Circulation 84(6):2325–2335CrossRefPubMed
3.
Zurück zum Zitat Schwartz ML, Gauvreau K, Geva T (2001) Predictors of outcome of biventricular repair in infants with multiple left heart obstructive lesions. Circulation 104(6):682–687CrossRefPubMed Schwartz ML, Gauvreau K, Geva T (2001) Predictors of outcome of biventricular repair in infants with multiple left heart obstructive lesions. Circulation 104(6):682–687CrossRefPubMed
4.
Zurück zum Zitat O’Byrne ML et al (2014) Morbidity in children and adolescents after surgical correction of interrupted aortic arch. Pediatr Cardiol 35(3):386–392CrossRefPubMed O’Byrne ML et al (2014) Morbidity in children and adolescents after surgical correction of interrupted aortic arch. Pediatr Cardiol 35(3):386–392CrossRefPubMed
5.
Zurück zum Zitat Mahle WT et al (2000) Neurodevelopmental outcome and lifestyle assessment in school-aged and adolescent children with hypoplastic left heart syndrome. Pediatrics 105(5):1082–1089CrossRefPubMed Mahle WT et al (2000) Neurodevelopmental outcome and lifestyle assessment in school-aged and adolescent children with hypoplastic left heart syndrome. Pediatrics 105(5):1082–1089CrossRefPubMed
6.
Zurück zum Zitat Feinstein JA et al (2012) Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol 59(1 Suppl):S1–S42CrossRefPubMed Feinstein JA et al (2012) Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol 59(1 Suppl):S1–S42CrossRefPubMed
7.
Zurück zum Zitat Anderson PA et al (2008) Contemporary outcomes after the Fontan procedure: a pediatric heart network multicenter study. J Am Coll Cardiol 52(2):85–98CrossRefPubMedPubMedCentral Anderson PA et al (2008) Contemporary outcomes after the Fontan procedure: a pediatric heart network multicenter study. J Am Coll Cardiol 52(2):85–98CrossRefPubMedPubMedCentral
8.
Zurück zum Zitat d’Udekem Y et al (2012) Predictors of survival after single-ventricle palliation: the impact of right ventricular dominance. J Am Coll Cardiol 59(13):1178–1185CrossRefPubMed d’Udekem Y et al (2012) Predictors of survival after single-ventricle palliation: the impact of right ventricular dominance. J Am Coll Cardiol 59(13):1178–1185CrossRefPubMed
9.
Zurück zum Zitat Murtuza B et al (2013) Results of orthotopic heart transplantation for failed palliation of hypoplastic left heart. Eur J Cardiothorac Surg 43(3):597–603CrossRefPubMed Murtuza B et al (2013) Results of orthotopic heart transplantation for failed palliation of hypoplastic left heart. Eur J Cardiothorac Surg 43(3):597–603CrossRefPubMed
10.
Zurück zum Zitat Puchalski MD et al (2004) Follow-up of aortic coarctation repair in neonates. J Am Coll Cardiol 44(1):188–191CrossRefPubMed Puchalski MD et al (2004) Follow-up of aortic coarctation repair in neonates. J Am Coll Cardiol 44(1):188–191CrossRefPubMed
11.
Zurück zum Zitat McElhinney DB et al (2005) Left heart growth, function, and reintervention after balloon aortic valvuloplasty for neonatal aortic stenosis. Circulation 111(4):451–458CrossRefPubMed McElhinney DB et al (2005) Left heart growth, function, and reintervention after balloon aortic valvuloplasty for neonatal aortic stenosis. Circulation 111(4):451–458CrossRefPubMed
12.
Zurück zum Zitat Serraf A et al (1999) Biventricular repair approach in ducto-dependent neonates with hypoplastic but morphologically normal left ventricle. J Am Coll Cardiol 33(3):827–834CrossRefPubMed Serraf A et al (1999) Biventricular repair approach in ducto-dependent neonates with hypoplastic but morphologically normal left ventricle. J Am Coll Cardiol 33(3):827–834CrossRefPubMed
13.
Zurück zum Zitat Kovalchin JP et al (1998) Echocardiographic hemodynamic and morphometric predictors of survival after two-ventricle repair in infants with critical aortic stenosis. J Am Coll Cardiol 32(1):237–244CrossRefPubMed Kovalchin JP et al (1998) Echocardiographic hemodynamic and morphometric predictors of survival after two-ventricle repair in infants with critical aortic stenosis. J Am Coll Cardiol 32(1):237–244CrossRefPubMed
14.
Zurück zum Zitat Minich LL et al (1997) Possibility of postnatal left ventricular growth in selected infants with non-apex-forming left ventricles. Am Heart J 133(5):570–574CrossRefPubMed Minich LL et al (1997) Possibility of postnatal left ventricular growth in selected infants with non-apex-forming left ventricles. Am Heart J 133(5):570–574CrossRefPubMed
15.
Zurück zum Zitat Colan SD et al (2006) Validation and re-evaluation of a discriminant model predicting anatomic suitability for biventricular repair in neonates with aortic stenosis. J Am Coll Cardiol 47(9):1858–1865CrossRefPubMed Colan SD et al (2006) Validation and re-evaluation of a discriminant model predicting anatomic suitability for biventricular repair in neonates with aortic stenosis. J Am Coll Cardiol 47(9):1858–1865CrossRefPubMed
16.
Zurück zum Zitat Blaufox AD et al (1998) Survival in neonatal biventricular repair of left-sided cardiac obstructive lesions associated with hypoplastic left ventricle. Am J Cardiol 82(9):1138–1140, A10CrossRefPubMed Blaufox AD et al (1998) Survival in neonatal biventricular repair of left-sided cardiac obstructive lesions associated with hypoplastic left ventricle. Am J Cardiol 82(9):1138–1140, A10CrossRefPubMed
17.
Zurück zum Zitat Tani LY et al (1999) Left heart hypoplasia and neonatal aortic arch obstruction: is the Rhodes left ventricular adequacy score applicable? J Thorac Cardiovasc Surg 118(1):81–86CrossRefPubMed Tani LY et al (1999) Left heart hypoplasia and neonatal aortic arch obstruction: is the Rhodes left ventricular adequacy score applicable? J Thorac Cardiovasc Surg 118(1):81–86CrossRefPubMed
18.
Zurück zum Zitat Tani LY et al (2000) Spectrum and influence of hypoplasia of the left heart in neonatal aortic coarctation. Cardiol Young 10(2):90–97CrossRefPubMed Tani LY et al (2000) Spectrum and influence of hypoplasia of the left heart in neonatal aortic coarctation. Cardiol Young 10(2):90–97CrossRefPubMed
19.
Zurück zum Zitat Alboliras ET et al (1999) Left ventricular growth in selected hypoplastic left ventricles: outcome after repair of coarctation of aorta. Ann Thorac Surg 68(2):549–555CrossRefPubMed Alboliras ET et al (1999) Left ventricular growth in selected hypoplastic left ventricles: outcome after repair of coarctation of aorta. Ann Thorac Surg 68(2):549–555CrossRefPubMed
20.
Zurück zum Zitat Lofland GK et al (2001) Critical aortic stenosis in the neonate: a multi-institutional study of management, outcomes, and risk factors. Congenital Heart Surgeons Society. J Thorac Cardiovasc Surg 121(1):10–27CrossRefPubMed Lofland GK et al (2001) Critical aortic stenosis in the neonate: a multi-institutional study of management, outcomes, and risk factors. Congenital Heart Surgeons Society. J Thorac Cardiovasc Surg 121(1):10–27CrossRefPubMed
21.
Zurück zum Zitat Lopez L et al (2010) Recommendations for quantification methods during the performance of a pediatric echocardiogram: a report from the Pediatric Measurements Writing Group of the American Society of Echocardiography Pediatric and Congenital Heart Disease Council. J Am Soc Echocardiogr 23(5):465–495 (quiz 576-7) CrossRefPubMed Lopez L et al (2010) Recommendations for quantification methods during the performance of a pediatric echocardiogram: a report from the Pediatric Measurements Writing Group of the American Society of Echocardiography Pediatric and Congenital Heart Disease Council. J Am Soc Echocardiogr 23(5):465–495 (quiz 576-7) CrossRefPubMed
22.
Zurück zum Zitat Pettersen MD et al (2008) Regression equations for calculation of z scores of cardiac structures in a large cohort of healthy infants, children, and adolescents: an echocardiographic study. J Am Soc Echocardiogr 21(8):922–934CrossRefPubMed Pettersen MD et al (2008) Regression equations for calculation of z scores of cardiac structures in a large cohort of healthy infants, children, and adolescents: an echocardiographic study. J Am Soc Echocardiogr 21(8):922–934CrossRefPubMed
23.
Zurück zum Zitat AHA et al (2006) AHA/ACC guidelines for secondary prevention for patients with coronary and other atherosclerotic vascular disease: 2006 update endorsed by the National Heart, Lung, and Blood Institute. J Am Coll Cardiol 47(10):2130–2139CrossRef AHA et al (2006) AHA/ACC guidelines for secondary prevention for patients with coronary and other atherosclerotic vascular disease: 2006 update endorsed by the National Heart, Lung, and Blood Institute. J Am Coll Cardiol 47(10):2130–2139CrossRef
24.
Zurück zum Zitat Baumgartner H et al (2009) Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice. J Am Soc Echocardiogr 22(1):1–23 (quiz 101–2) CrossRefPubMed Baumgartner H et al (2009) Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice. J Am Soc Echocardiogr 22(1):1–23 (quiz 101–2) CrossRefPubMed
25.
Zurück zum Zitat Harris PA et al (2009) Research electronic data capture (REDCap)—a metadata-driven methodology and workflow process for providing translational research informatics support. J Biomed Inform 42(2):377–381CrossRefPubMed Harris PA et al (2009) Research electronic data capture (REDCap)—a metadata-driven methodology and workflow process for providing translational research informatics support. J Biomed Inform 42(2):377–381CrossRefPubMed
26.
Zurück zum Zitat Ghanayem NS et al (2010) Perioperative monitoring in high-risk infants after stage 1 palliation of univentricular congenital heart disease. J Thorac Cardiovasc Surg 140(4):857–863CrossRefPubMed Ghanayem NS et al (2010) Perioperative monitoring in high-risk infants after stage 1 palliation of univentricular congenital heart disease. J Thorac Cardiovasc Surg 140(4):857–863CrossRefPubMed
27.
Zurück zum Zitat Gray RG et al (2013) Long-term follow-up of neonatal coarctation and left-sided cardiac hypoplasia. Am J Cardiol 111(9):1351–1354CrossRefPubMed Gray RG et al (2013) Long-term follow-up of neonatal coarctation and left-sided cardiac hypoplasia. Am J Cardiol 111(9):1351–1354CrossRefPubMed
28.
Zurück zum Zitat Nicholson GT et al (2016) Late outcomes in children with Shone’s complex: a single-centre, 20-year experience. Cardiol Young 27:1–9 Nicholson GT et al (2016) Late outcomes in children with Shone’s complex: a single-centre, 20-year experience. Cardiol Young 27:1–9
29.
Zurück zum Zitat Krauser DG, Rutkowski M, Phoon CK (2000) Left ventricular volume after correction of isolated aortic coarctation in neonates. Am J Cardiol 85(7):904–907, A10CrossRefPubMed Krauser DG, Rutkowski M, Phoon CK (2000) Left ventricular volume after correction of isolated aortic coarctation in neonates. Am J Cardiol 85(7):904–907, A10CrossRefPubMed
30.
Zurück zum Zitat Zahorec M et al (2014) Natural history of newborn with borderline small left heart without interatrial communication. World J Pediatr Congenit Heart Surg 5(1):100–104CrossRefPubMed Zahorec M et al (2014) Natural history of newborn with borderline small left heart without interatrial communication. World J Pediatr Congenit Heart Surg 5(1):100–104CrossRefPubMed
31.
Zurück zum Zitat Ruffer A et al (2015) Aortic arch obstruction neonates with biventricular physiology: left-open compared to closed inter-atrial communication during primary repair—a retrospective study. J Cardiothorac Surg 10:53CrossRefPubMedPubMedCentral Ruffer A et al (2015) Aortic arch obstruction neonates with biventricular physiology: left-open compared to closed inter-atrial communication during primary repair—a retrospective study. J Cardiothorac Surg 10:53CrossRefPubMedPubMedCentral
32.
Zurück zum Zitat Levine JC et al (2001) The risk of having additional obstructive lesions in neonatal coarctation of the aorta. Cardiol Young 11(1):44–53CrossRefPubMed Levine JC et al (2001) The risk of having additional obstructive lesions in neonatal coarctation of the aorta. Cardiol Young 11(1):44–53CrossRefPubMed
Metadaten
Titel
The Infant with Aortic Arch Hypoplasia and Small Left Heart Structures: Echocardiographic Indices of Mitral and Aortic Hypoplasia Predicting Successful Biventricular Repair
verfasst von
Jennifer M. Plymale
Peter C. Frommelt
Melodee Nugent
Pippa Simpson
James S. Tweddell
Amanda J. Shillingford
Publikationsdatum
04.07.2017
Verlag
Springer US
Erschienen in
Pediatric Cardiology / Ausgabe 6/2017
Print ISSN: 0172-0643
Elektronische ISSN: 1432-1971
DOI
https://doi.org/10.1007/s00246-017-1661-2

Weitere Artikel der Ausgabe 6/2017

Pediatric Cardiology 6/2017 Zur Ausgabe

Update Kardiologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.