Ventricular function and ventriculo-arterial coupling after palliation of hypoplastic left heart syndrome: A comparative study with Fontan patients with LV morphology

2017 ◽  
Vol 227 ◽  
pp. 691-697 ◽  
Author(s):  
J. Logoteta ◽  
C. Ruppel ◽  
J.H. Hansen ◽  
G. Fischer ◽  
K. Becker ◽  
...  
Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Christoph Haller ◽  
Devin Chetan ◽  
Matthew Doyle ◽  
Arezou Saedi ◽  
Rachel Parker ◽  
...  

Objectives: The interdigitating technique in aortic arch reconstruction in hypoplastic left heart syndrome and variants (HLHS) is very effective to minimize the recoarctation rate. Little is known on the aortic arch’s growth characteristics and the resulting clinical impact. Methods: 139 patients with HLHS underwent staged palliation between 2007 and 2014. 72 patients who underwent Norwood arch reconstruction with the interdigitating technique were included. Dimensions of the ascending aorta (AA), transverse arch (TA), isthmus (IA) and descending aorta (DA) in pre-stage II (P1, n=50) and pre-Fontan (P2, n=21) angiograms were measured and geometry and growth characteristics of the aortic arches were analyzed. Correlations between the aortic dimensions and clinical outcomes were assessed. Results: There were significant increases in diameters in all segments between P1 and P2 (p < .0005). The z-scores in AA, TA and IA were unchanged between P1 and P2 (p = .931/.425/.121), but increased significantly in DA at P2 (p = .039). The percent increase in diameters were comparable among 4 segments (mean, 146% in IA, 144 in DA, p=.648). There were correlations in dimensions and z-scores between P1 and P2 in AA (p = .029/.013) and TA (p = .001/ < .0005), but no correlations were found in IA (p = .140/.747) and DA (p = .075/.432). The most significant tapering in the arch dimension occurred between TA and IA in both time points (P1, 67.3% vs. P2, 61.1%, p=.303). The reverse coarctation index (TA/IA ratio) at P1 (r = .381, p = .042), but not coarctation index (CoAI, IA/DA ratio) at P1 (p = .774) had a significant correlation with post-stage II ventricular function. Balloon dilatation for recoarctation was needed in 2 (2.7%) patients prior to stage II palliation. CoAI at P1 was a predictor for ventricular dysfunction at latest follow-up (p=.017). Conclusions: Aortic arch growth after interdigitating reconstruction in HLHS is substantial and relatively constant. The isthmus growth is proportional to other segments. Overall reintervention rate for recoarctation is exceptionally low. CoAI prior to stage II palliation may be associated with long-term ventricular function.


Author(s):  
Dai Asada ◽  
Yoko Kawai ◽  
Yoshinobu Maeda ◽  
Masaaki Yamagishi

Abstract A male neonate presented with the aortic/mitral stenotic variant of hypoplastic left heart syndrome, wherein the suprasystemic left ventricular pressure and relatively large left ventricle had shifted the intraventricular septum. Despite bilateral pulmonary artery banding, the stroke volume was difficult to maintain owing to the compressed right ventricle, causing heart failure symptoms. Percutaneous balloon aortic valvuloplasty decreased the left ventricular pressure, restoring the right ventricular function. Norwood procedure with mitral valve closure after catheter intervention reduced the left ventricular size and improved the right ventricular function. This paper refers to the potential of mitral valve closure for hypoplastic left heart syndrome.


2008 ◽  
Vol 86 (3) ◽  
pp. 869-874 ◽  
Author(s):  
Luis Alesandro Larrazabal ◽  
Elif Seda Selamet Tierney ◽  
David W. Brown ◽  
Kimberlee Gauvreau ◽  
Vladimiro L. Vida ◽  
...  

2015 ◽  
Vol 26 (5) ◽  
pp. 867-875 ◽  
Author(s):  
Daisuke Kobayashi ◽  
Girija Natarajan ◽  
Daniel R. Turner ◽  
Thomas J. Forbes ◽  
Ralph E. Delius ◽  
...  

AbstractObjectiveThe effect of Hybrid stage 1 palliation for hypoplastic left heart syndrome on right ventricular function is unknown. We sought to compare right ventricular function in normal neonates and those with hypoplastic left heart syndrome before Hybrid palliation and to assess the effect of Hybrid palliation on right ventricular function, using the right ventricular myocardial performance index and the ratio of systolic and diastolic durations.MethodsWe carried out a retrospective review of echocardiographic data on 23 infants with hypoplastic left heart syndrome who underwent Hybrid palliation and 35 normal controls. Data were acquired before Hybrid and after Hybrid palliation – post 1, 0–4 days; post 2, 1 week; post 3, 2–3 weeks; post 4, 1–1.5 months following Hybrid palliation.ResultsMyocardial performance index and ratio of systolic and diastolic durations were higher in the pre-Hybrid hypoplastic left heart syndrome group (n=23) – 0.47±0.16 versus 0.25±0.07, p<0.001; 1.59±0.44 versus 1.09±0.14, p<0.0001 – compared with controls (n=35). There was no significant change in the myocardial performance index at any of the post-Hybrid time points. Ratio of systolic and diastolic durations increased significantly 2 weeks after Hybrid – post 3: 2.08±0.62 and post 4: 2.21±0.45 versus pre: 1.59±0.44, p=0.043 and 0.003. There were no significant differences in parameters between sub-groups of infants who died (n=10) and survivors (n=13).ConclusionsRight ventricular myocardial performance index and ratio of systolic and diastolic durations were significantly higher in infants with hypoplastic left heart syndrome before intervention compared with controls. The ratio of systolic and diastolic durations increased significantly 2 weeks after Hybrid palliation. Our data suggest that infants with hypoplastic left heart syndrome have right ventricular dysfunction before intervention, which worsens over 2 weeks after Hybrid palliation.


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