Myocardial Perfusion in Hypoplastic Left Heart Syndrome
| Autor: | Jens Scheewe, Carsten Rickers, Hans-Heiner Kramer, Michael Silberbach, Dominik Daniel Gabbert, Philip Wegner, Arash Kheradvar, Inga Voges, Erin J. Madriago, Tim Attmann, Michael Jerosch-Herold |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
Male
Myocardial Ischemia Coronary microcirculation Cardiovascular Hypoplastic left heart syndrome Fontan circulation Infant Mortality Hypoplastic Left Heart Syndrome magnetic resonance imaging Prospective Studies Child Pediatric screening and diagnosis medicine.diagnostic_test Doppler Myocardial Perfusion Imaging Echocardiography Doppler Detection medicine.anatomical_structure Heart Disease Cine Echocardiography Child Preschool Cardiology heart defects Female Cardiology and Cardiovascular Medicine Perfusion 4.2 Evaluation of markers and technologies medicine.medical_specialty Clinical Sciences Magnetic Resonance Imaging Cine Myocardial perfusion imaging Rare Diseases Internal medicine Coronary Circulation medicine Humans Radiology Nuclear Medicine and imaging Preschool Heart Disease - Coronary Heart Disease business.industry Microcirculation congenital Magnetic resonance imaging medicine.disease Cardiovascular System & Hematology Ventricle Oxygen Saturation business Follow-Up Studies |
| Zdroj: | Circulation. Cardiovascular imaging, vol 14, iss 10 |
| ISSN: | 1942-0080 |
| Popis: | Background: The status of the systemic right ventricular coronary microcirculation in hypoplastic left heart syndrome (HLHS) is largely unknown. It is presumed that the systemic right ventricle’s coronary microcirculation exhibits unique pathophysiological characteristics of HLHS in Fontan circulation. The present study sought to quantify myocardial blood flow by cardiac magnetic resonance imaging and evaluate the determinants of microvascular coronary dysfunction and myocardial ischemia in HLHS. Methods: One hundred nineteen HLHS patients (median age, 4.80 years) and 34 healthy volunteers (median age, 5.50 years) underwent follow-up cardiac magnetic resonance imaging ≈1.8 years after total cavopulmonary connection. Right ventricle volumes and function, myocardial perfusion, diffuse fibrosis, and late gadolinium enhancement were assessed in 4 anatomic HLHS subtypes. Myocardial blood flow (MBF) was quantified at rest and during adenosine-induced hyperemia. Coronary conductance was estimated from MBF at rest and catheter-based measurements of mean aortic pressure (n=99). Results: Hyperemic MBF in the systemic ventricle was lower in HLHS compared with controls (1.89±0.57 versus 2.70±0.84 mL/g per min; P P =0.446). Independent risk factors for a reduced hyperemic MBF were an HLHS subtype with mitral stenosis and aortic atresia ( P =0.017), late gadolinium enhancement ( P =0.042), right ventricular diastolic dysfunction ( P =0.005), and increasing age at total cavopulmonary connection ( P =0.022). The coronary conductance correlated negatively with systemic blood oxygen saturation (r, −0.29; P =0.02). The frequency of late gadolinium enhancement increased with age at total cavopulmonary connection ( P =0.014). Conclusions: The coronary microcirculation of the systemic ventricle in young HLHS patients shows significant differences compared with controls. These hypothesis-generating findings on HLHS-specific risk factors for microvascular dysfunction suggest a potential benefit from early relief of frank cyanosis by total cavopulmonary connection. |
| Databáze: | OpenAIRE |
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