Reduced ascending aorta distensibility relates to adverse ventricular mechanics in patients with hypoplastic left heart syndrome: Noninvasive study using wave intensity analysis

Biglino, Giovanni; Schievano, Silvia; Steeden, Jennifer A.; Ntsinjana, Hopewell; Baker, Catriona; Khambadkone, Sachin; Leval, Marc R. de; Hsia, Tain‐Yen; Taylor, Andrew M.; Giardini, Alessandro

doi:10.1016/j.jtcvs.2012.08.028

Cited by 54 publications

(48 citation statements)

References 25 publications

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“…These results indicate that SV dysfunction seems relatively unrelated to volume overload but is probably more affected by afterload conditions, as previously described [17][18][19]. Based on our results and current studies that investigated the effect of additional volume overload through aortopulmonary shunt implantation in BCPC-patients [20,21], it is unlikely that a mild or moderate amount of APC flow leads to myocardial dysfunction.…”

Section: Discussionsupporting

confidence: 86%

Aortopulmonary Collateral Flow is Related to Pulmonary Artery Size and Affects Ventricular Dimensions in Patients After the Fontan Procedure

Latus¹,

Gummel²,

Diederichs³

et al. 2013

Thorac cardiovasc Surg

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Background: Aortopulmonary collaterals (APCs) are frequently found in patients with a single-ventricle (SV) circulation. However, knowledge about the clinical significance of the systemic-to-pulmonary shunt flow in patients after the modified Fontan procedure and its potential causes is limited. Accordingly, the aim of our study was to detect and quantify APC flow using cardiovascular magnetic resonance (CMR) and assess its impact on SV volume and function as well as to evaluate the role of the size of the pulmonary arteries in regard to the development of APCs.

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Section: Discussionsupporting

confidence: 86%

Aortopulmonary Collateral Flow is Related to Pulmonary Artery Size and Affects Ventricular Dimensions in Patients After the Fontan Procedure

Latus¹,

Gummel²,

Diederichs³

et al. 2013

Thorac cardiovasc Surg

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“…Reduced aortic distensibility and deficiency in ventricular arterial coupling in single-ventricle patients may partly explain this finding. 10 One could also suspect that a low threshold for arch reintervention should be applied in patients with single ventricles. It was surprising to note that common atrioventricular morphology did not predict any adverse outcome and one could wonder whether this fact is not a consequence of a progressively more aggressive management of atrioventricular valve regurgitation.…”

Section: Discussionmentioning

confidence: 99%

Redefining Expectations of Long-Term Survival After the Fontan Procedure

et al. 2014

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Background— The life expectancy of patients undergoing a Fontan procedure is unknown. Methods and Results— Follow-up of all 1006 survivors of the 1089 patients who underwent a Fontan procedure in Australia and New Zealand was obtained from a binational population-based registry including all pediatric and adult cardiac centers. There were 203 atriopulmonary connections (AP; 1975–1995), 271 lateral tunnels (1988–2006), and 532 extracardiac conduits (1997–2010). The proportion with hypoplastic left heart syndrome increased from 1/173 (1%) before 1990 to 80/500 (16%) after 2000. Survival at 10 years was 89% (84%–93%) for AP and 97% (95% confidence interval [CI], 94%–99%) for lateral tunnels and extracardiac conduits. The longest survival estimate was 76% (95% CI, 67%–82%) at 25 years for AP. AP independently predicted worse survival compared with extracardiac conduits (hazard ratio, 6.2; P <0.001; 95% CI, 2.4–16.0). Freedom from failure (death, transplantation, takedown, conversion to extracardiac conduits, New York Heart Association III/IV, or protein-losing enteropathy/plastic bronchitis) 20 years after Fontan was 70% (95% CI, 63%–76%). Hypoplastic left heart syndrome was the primary predictor of Fontan failure (hazard ratio, 3.8; P <0.001; 95% CI, 2.0–7.1). Ten-year freedom from failure was 79% (95% CI, 61%–89%) for hypoplastic left heart syndrome versus 92% (95% CI, 87%–95%) for other morphologies. Conclusions— The long-term survival of the Australia and New Zealand Fontan population is excellent. Patients with an AP Fontan experience survival of 76% at 25 years. Technical modifications have further improved survival. Patients with hypoplastic left heart syndrome are at higher risk of failure. Large, comprehensive registries such as this will further improve our understanding of late outcomes after the Fontan procedure.

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“…The fate of the reconstructed aorta in HLHS has been assessed in previous studies 1, 2, 3. Cardis et al found increased stiffness and decreased distensibility in the reconstructed aortic arch by using echocardiography 3.…”

Section: Introductionmentioning

confidence: 99%

“…Cardis et al found increased stiffness and decreased distensibility in the reconstructed aortic arch by using echocardiography 3. In a cardiac magnetic resonance imaging (CMR) study of a small cohort of HLHS patients (n=10), Biglino et al reported greater aortic pulse wave velocity (PWV) and reduced distensibility of the ascending aorta 2. In addition, they found lower ascending aortic wave intensity and energy, suggesting abnormal ventricular–vascular coupling 2.…”

Section: Introductionmentioning

confidence: 99%

“…In a cardiac magnetic resonance imaging (CMR) study of a small cohort of HLHS patients (n=10), Biglino et al reported greater aortic pulse wave velocity (PWV) and reduced distensibility of the ascending aorta 2. In addition, they found lower ascending aortic wave intensity and energy, suggesting abnormal ventricular–vascular coupling 2. We previously demonstrated in 40 children with HLHS that the reconstructed aortic arch after Norwood procedure is significantly dilated and has reduced distensibility with negative effects on the function of the systemic right ventricle 1.…”

Section: Introductionmentioning

confidence: 99%

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Frequent Dilatation of the Descending Aorta in Children With Hypoplastic Left Heart Syndrome Relates to Decreased Aortic Arch Elasticity

Voges

Jerosch‐Herold

Wegner

et al. 2015

JAHA

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BackgroundPatients with hypoplastic left heart syndrome after a Norwood operation show dilatation and reduced distensibility of the reconstructed proximal aorta. Cardiac magnetic resonance imaging (CMR) and angiographic examinations indicate that the native descending aorta (DAo) is also dilated, but this has not been studied in detail.Methods and ResultsSeventy‐nine children with hypoplastic left heart syndrome in Fontan circulation (aged 6.3±3.2 years) and 18 control participants (aged 6.8±2.4 years) underwent 3.0‐tesla CMR. Gradient‐echo cine and phase‐contrast imaging was applied to measure cross‐sectional areas (CSAs), distensibility, pulse wave velocity, and the incremental elastic modulus of the thoracic aorta. CSA of the DAo in patients was also compared with published percentiles for aortic CSA. Patients had significantly larger CSA of the DAo at the level of pulmonary artery bifurcation (229.1±97.2 versus 175.7±24.3 mm/m2, P=0.04) and the diaphragm (196.2±66.0 versus 142.6±16.7 mm/m2, P<0.01). In 41 patients (52%), CSA of the DAo was >95th percentile level for control participants, and the incremental elastic modulus of the aortic arch and the DAo was higher than in patients with normal CSAs (arch: 90.1±64.3 versus 45.6±38.9 m/s; DAo: 86.3±53.7 versus 47.1±47.6 m/s; P<0.01). Incremental elastic modulus of the aortic arch and the DAo correlated with the CSA of the DAo (arch: r=0.5; DAo: r=0.49; P<0.01).ConclusionsChildren with hypoplastic left heart syndrome frequently show dilatation of their DAo associated with increased stiffness of the aortic arch. Higher aortic impedance increases the afterload of the systemic circulation and likely contributes to the burden of the systemic right ventricle.

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Reduced ascending aorta distensibility relates to adverse ventricular mechanics in patients with hypoplastic left heart syndrome: Noninvasive study using wave intensity analysis

Cited by 54 publications

References 25 publications

Aortopulmonary Collateral Flow is Related to Pulmonary Artery Size and Affects Ventricular Dimensions in Patients After the Fontan Procedure

Aortopulmonary Collateral Flow is Related to Pulmonary Artery Size and Affects Ventricular Dimensions in Patients After the Fontan Procedure

Redefining Expectations of Long-Term Survival After the Fontan Procedure

Frequent Dilatation of the Descending Aorta in Children With Hypoplastic Left Heart Syndrome Relates to Decreased Aortic Arch Elasticity

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