scholarly journals OP12.07: Velocity vector imaging of longitudinal function, strain and strain rate in the right ventricle of the fetus with hypoplastic left heart syndrome

2010 ◽  
Vol 36 (S1) ◽  
pp. 86-86
Author(s):  
C. Saunders ◽  
K. Jayakumar ◽  
A. Hoyer
Keyword(s):  
Right Ventricle ◽  
Strain Rate ◽  
Hypoplastic Left Heart Syndrome ◽  
Velocity Vector ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Longitudinal Function ◽  
Strain And Strain Rate ◽  
The Right ◽  
Left Heart Syndrome

Pathological changes and myocardial remodelling related to the mode of shunting following surgical palliation for hypoplastic left heart syndrome

2008 ◽  
Vol 18 (4) ◽  
pp. 415-422 ◽  
Author(s):  
Massimo A. Padalino ◽  
Chiara Castellani ◽  
Silvia Toffoli ◽  
Mila Della Barbera ◽  
Ornella Milanesi ◽  
...  
Keyword(s):  
Extracellular Matrix ◽  
Right Ventricle ◽  
Hypoplastic Left Heart Syndrome ◽  
Pulmonary Arteries ◽  
Left Heart ◽  
Pulmonary Shunt ◽  
Hypoplastic Left Heart ◽  
Surgical Palliation ◽  
The Right ◽  
Left Heart Syndrome

AbstractBackgroundThe modification of placing the shunt from the right ventricle to the pulmonary arteries, also known as Sano procedure, has allegedly improved results over the short term in surgical palliation of hypoplastic left heart syndrome with the Norwood procedure. With this in mind, we reviewed autopsied specimens from neonates and children who did not survive after either a classic arterio-pulmonary shunt, or the modified procedure with the shunt placed from the right ventricle to the pulmonary arteries, so as to evaluate the pathological substrates of the remodelling of the systemic right ventricle, assessing any differences induced by the 2 techniques.MethodsWe obtained the hearts from 11 patients with neonatal diagnosis of hypoplastic left heart syndrome who died after the first or second stages of the Norwood sequence of operations, comparing them with 6 normal hearts matched for age and weight. Macroscopic, microscopic and morphometric analysis were performed on each specimen, evaluating the diameter of the myocytes, extracellular matrix remodelling in terms of fibrosis and type of collagen, and vascularization in terms of capillary density.ResultsHypertrophy of the myocytes was significantly increased in the hearts from patients having either a classic arterio-pulmonary or the ventriculo-pulmonary modification of the shunt compared to controls (p < 0.05). Myocardial fibrosis was increased in those having a shunt placed from the right ventricle to the pulmonary arteries when compared to the other 2 groups. The ratio of collagen I to collagen III was similar in those undergoing a classic arterio-pulmonary shunt compared to controls (0.94), but was lower in those having a shunt placed from the right ventricle to the pulmonary arteries (0.61), with an increase in collagen type III. The density of capillaries was lower in those who had undergone a classic arterial shunt when compared to the others.ConclusionWe have shown greater remodelling of the ventricular myocardial extracellular matrix in patients having a shunt from the right ventricle to the pulmonary arteries when compared to those having a classic arterio-pulmonary shunt, with this remodelling progressing even after the neonatal period. This may influence a later suboptimal ventricular performance.

The management of hypoplastic left heart syndrome with a right aortic arch

2006 ◽  
Vol 16 (5) ◽  
pp. 504-506 ◽  
Author(s):  
Gregory H. Tatum ◽  
Victor O. Morell ◽  
Sang C. Park
Keyword(s):  
Right Ventricle ◽  
Aortic Arch ◽  
Hypoplastic Left Heart Syndrome ◽  
Pulmonary Arteries ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Cavopulmonary Anastomosis ◽  
Reliable Source ◽  
The Right ◽  
Left Heart Syndrome

The combination of hypoplastic left heart syndrome and a right-sided aortic arch is extremely rare and lethal. To the best of our knowledge, no patient with this combination has previously been reported as surviving initial palliation. The anatomic variant is associated with abnormalities in the arteries branching from the aortic arch, making it difficult to construct a reliable source of flow of blood to the lungs. We present here a patient with this combination who survived an initial Damus-Kay-Stansel procedure combined with placement of a conduit from the right ventricle to the pulmonary arteries, and who has subsequently undergone a successful bidirectional cavopulmonary anastomosis. We believe that the conduit placed from the right ventricle provides the most reliable source of flow of blood to the lungs at the time of initial palliation in this usual combination of cardiac lesions.

scholarly journals Decreasing Interstage Mortality After the Norwood Procedure: A 30‐Year Experience

2020 ◽  
Vol 9 (19) ◽  
Author(s):  
Michelle Kaplinski ◽  
Richard F. Ittenbach ◽  
Mallory L. Hunt ◽  
Donna Stephan ◽  
Shobha S. Natarajan ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Right Ventricle ◽  
Hypoplastic Left Heart Syndrome ◽  
Monitoring Program ◽  
Norwood Procedure ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Interstage Mortality ◽  
The Right ◽  
Left Heart Syndrome

Background The superior cavo‐pulmonary connection was introduced at our institution in 1988 for infants undergoing surgery for hypoplastic left heart syndrome. Patients with hypoplastic left heart syndrome remain at high risk for mortality in the time period between the Norwood procedure and the superior cavo‐pulmonary connection. The primary objectives of this study were to compare interstage mortality across 4 eras and analyze factors that may impact interstage mortality. Methods and Results Patients with hypoplastic left heart syndrome who underwent the Norwood procedure, were discharged from the hospital, and were eligible for superior cavo‐pulmonary connection between January 1, 1988, and December 31, 2017, were included. The study period was divided into 4 eras based on changes in operative or medical management. Mortality rates were estimated with 95% CIs. Adjusted and unadjusted logistic regression models were used to identify risk factors for mortality. There were 1111 patients who met the inclusion criteria. Overall, interstage mortality was 120/1111 (10.8%). Interstage mortality was significantly lower in era 4 relative to era 1 (4.6% versus 13.4%; P =0.02) during the time that age at the superior cavo‐pulmonary connection was the lowest (135 days; P <0.01) and the interstage monitoring program was introduced. In addition, use of the right ventricle to pulmonary artery shunt was associated with decreased interstage mortality ( P =0.02) and was more routinely practiced in era 4. Conclusions During this 30‐year experience, the risk of interstage mortality decreased significantly in the most recent era. Factors that coincide with this finding include younger age at superior cavo‐pulmonary connection, introduction of an interstage monitoring program, and increased use of the right ventricle to pulmonary artery shunt.

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