Extensive Hilum to Hilum Pulmonary Artery Plasty in Stage II Palliation of Hypoplastic Left Heart Syndrome

2020 ◽  
Author(s):  
Osami Honjo ◽  
Shuhua Luo
Keyword(s):  
Pulmonary Artery ◽  
Hypoplastic Left Heart Syndrome ◽  
Stage Ii ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome

Abstract 19315: Geometry, Reintervention, and Growth Patterns in the Reconstructed Aortic Arch With Interdigitating Technique in Patients With Hypoplastic Left Heart Syndrome and Variants

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Christoph Haller ◽  
Devin Chetan ◽  
Matthew Doyle ◽  
Arezou Saedi ◽  
Rachel Parker ◽  
...  
Keyword(s):  
Aortic Arch ◽  
Ventricular Function ◽  
Hypoplastic Left Heart Syndrome ◽  
Growth Characteristics ◽  
Stage Ii ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Z Scores ◽  
Arch Reconstruction ◽  
Left Heart Syndrome

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.

scholarly journals Pulmonary artery configuration after palliative operations for hypoplastic left heart syndrome

1989 ◽  
Vol 97 (6) ◽  
pp. 878-885 ◽  
Author(s):  
Ernerio T. Alboliras ◽  
Alvin J. Chin ◽  
Gerald Barber ◽  
J. Gregg Helton ◽  
John D. Pigott ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Hypoplastic Left Heart Syndrome ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome

Hypoplastic left heart syndrome with anomalous origin of right pulmonary artery

2020 ◽  
pp. 021849232095506
Author(s):  
Ashish Mishra ◽  
Bhavik Champaneri ◽  
Yashpal Rana ◽  
Gaurav Singh ◽  
Senthilraj Thangasami ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Hypoplastic Left Heart Syndrome ◽  
Venous Drainage ◽  
Anomalous Origin ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Right Pulmonary Artery ◽  
Complex Forms ◽  
The Right ◽  
Left Heart Syndrome

Classic hypoplastic left heart syndrome is a rare but fatal congenital heart disease associated with variable underdevelopment of the left side of the heart. Complex forms of hypoplastic left heart syndrome have been reported to coexist with anomalous pulmonary venous drainage, transposition of the great arteries, or pulmonary valve dysplasia. We report a case of hypoplastic left heart syndrome with anomalous origin of the right pulmonary artery from the ascending aorta a rare association not reported in the literature. Preoperative comprehensive echocardiography is essential for diagnosis and accurate recognition of such rare anatomic variations.

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