Hybrid Pulmonary Artery Stenting at the Bidirectional Glenn or Fontan Operation in Patients With Single Ventricle Congenital Heart Disease

2016 ◽  
Vol 7 (3) ◽  
pp. 299-305 ◽  
Author(s):  
Matthew C. Schwartz ◽  
William M. DeCampli ◽  
Kamal Pourmoghadam ◽  
Karen Iacono ◽  
David Nykanen
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Artery ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Fontan Operation ◽  
Bidirectional Glenn

scholarly journals Transplantation for Congenital Heart Disease: Focus on the Impact of Functionally Univentricular Versus Biventricular Circulation

2021 ◽  
Vol 12 (3) ◽  
pp. 352-359
Author(s):  
Kyle W. Riggs ◽  
John T. Broderick ◽  
Nina Price ◽  
Clifford Chin ◽  
Farhan Zafar ◽  
...  
Keyword(s):  
Risk Factors ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Fontan Operation ◽  
Blood Type ◽  
Abo Blood Type ◽  
The Impact ◽  
Fontan Patients

Background: Varying single center data exist regarding the posttransplant outcomes of patients with single ventricle circulation, particularly following the Fontan operation. We sought to better elucidate these results in patients with congenital heart disease (CHD) through combining two national databases. Methods: The United Network for Organ Sharing (UNOS) transplantation database was merged with the Pediatric Health Information System (PHIS), an administrative database with 71% of UNOS patients matched. Patients undergoing transplantation at a PHIS hospital from 2006 to 2017 were categorized as single ventricle or biventricular strategy based on their diagnoses and procedures in 90% of patients. When known, single ventricle patients were further analyzed by their palliative stage post-Glenn or post-Fontan (known in 31%). Results: A total of 1,517 CHD transplantations were identified, 67% with single ventricle strategy (1,016). Single ventricle, biventricular, and indeterminate patients had similar survival (log-rank P > .1). Risk factors for mortality in patients with CHD were extracorporeal membrane oxygenation (ECMO) support at transplant (hazard: 2.27), ABO blood type incompatibility (hazard: 1.61), African American recipient (hazard 1.42), and liver dysfunction (hazard 1.29). A total of 130 confirmed Fontan and 185 confirmed bidirectional Glenn patients underwent transplantation, each with survival equivalent to biventricular patients (log-rank P > .500). For Fontan patients, renal dysfunction (hazard: 5.40) and transplant <1 year after Fontan (hazard 2.82) were found to be associated with mortality. Conclusions: Single ventricle patients, as a group, experience similar outcomes as biventricular patients with CHD undergoing transplantation, and this extends to Fontan patients. Risk factors for mortality correlate with end-organ dysfunction as well as race and ABO blood type incompatibility in the CHD population.

scholarly journals Role of Transient Elastography to Stage Fontan-Associated Liver Disease (FALD) in Adults with Single Ventricle Congenital Heart Disease Correction

2021 ◽  
Vol 8 (10) ◽  
pp. 117
Author(s):  
Liliana Chemello ◽  
Massimo Padalino ◽  
Chiara Zanon ◽  
Luisa Benvegnu’ ◽  
Roberta Biffanti ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Platelet Count ◽  
Heart Disease ◽  
Liver Disease ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Transient Elastography ◽  
Fontan Operation ◽  
Liver Stiffness ◽  
Morbidity And Mortality

Fontan-associated liver disease (FALD) is an arising clinical entity that can occur long after a successful Fontan operation for correction of single ventricle (SV) congenital heart disease (CHD). Occurrence of FALD is characterized by liver cirrhosis and other hepatic complications, and determinates an increased morbidity and mortality. Currently, there is no consensus on how to stage FALD. We report here our experience by an observational study in 52 patients with SV-CHD after Fontan operation that were recruited through a period of 36 ± 9.3 months. All cases underwent lab tests and liver and cardiac imaging evaluation, including liver stiffness (LS) measurement by transient elastography (TE) (FibroScan®). According to selective criteria for liver disease, we identified 23/43 (53.5%) cases with advanced FALD that showed: older age (p < 0.05), larger hepatic and cava veins diameter (p < 0.05), worsened NYHA class (p < 0.05), abnormal lymphocytes (p < 0.01), platelet count (p < 0.05), and GGT, prothrombin time (INR), albumin and cystatin C levels (p < 0.05), with respect to cases without advanced FALD. LS values were significantly increased in cases with advanced FALD, at cut-off values higher than 22 kPa (p < 0.001). LS, and its combined score with spleen diameter and platelet count (LSPS) successfully helped to detect 100% of cases with portal hypertension (p < 0.001). In conclusion, LS can be effective to stage FALD and to uncover cases with severe risk of complications, avoiding higher morbidity and mortality related to advanced FALD.

scholarly journals Identification and characterization of a novel ELN mutation in congenital heart disease with pulmonary artery stenosis

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Cuilan Hou ◽  
Junmin Zheng ◽  
Wei liu ◽  
Lijian Xie ◽  
Xiaomin Sun ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Artery ◽  
Molecular Mechanism ◽  
Protein Level ◽  
Congenital Heart ◽  
Artery Stenosis ◽  
Pulmonary Artery Stenosis ◽  
Functional Component ◽  
Functional Changes

AbstractCongenital heart defects, one of the most common birth defects, affect approximately 1% of live birth globally and remain the leading cause of infant mortality in developed countries. Utilizing the pathogenicity score and inheritance mode from whole exome sequencing results, a heterozygous mutation (NM_001278939.1: c.1939G>T, p.Gly647Ter) in elastin (ELN) was identified among 6,440 variants in a female proband born with an atrial septal defect accompanied by pulmonary artery stenosis. Results of RT-PCR showed that the mutation (NM_001278939.1: c.1939G>T, p.Gly647Ter) did not affect the expression levels of ELN mRNA but increased protein level. The content of ELN truncate (functional component) was significantly lower in both the intracellular and extracellular compartments after mutation. These results indicate that the ELN mutation (NM_001278939.1: c.1939G>T, p.Gly647Ter) affected the protein truncate, which may be a functional component of ELN and play crucial roles for this pedigree. Here we report of an ELN heterozygous variant associated with congenital heart disease accompanied with pulmonary artery stenosis, which is less common. Based on our results, we speculate that this may be the main molecular mechanism underlying the mutation-led functional changes, and propose that the decrease of ELN protein level may cause this pedigree vascular abnormality, especially pulmonary artery stenosis, and reinforce the view that ELN insufficiency is the primary cause of these vascular lesions. This may be the main molecular mechanism underlying the mutation-led functional changes. Thus, systematic analysis not only enables us to better understand the etiology of this disease but also contributes to clinical and prenatal diagnosis.

scholarly journals Anomalous Origin of the Left Anterior Descending Coronary Artery in an Adult

2021 ◽  
Vol 10 (01) ◽  
pp. e9-e10
Author(s):  
Keisuke Shibagaki ◽  
Chikara Shiiku ◽  
Hiroyuki Kamiya ◽  
Yoichi Kikuchi
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Coronary Artery ◽  
Pulmonary Artery ◽  
Congenital Heart ◽  
Left Coronary Artery ◽  
Internal Thoracic Artery ◽  
Artery Bypass ◽  
Anomalous Origin ◽  
Bypass Grafting

AbstractAn anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease. Among the variants, an anomalous origin of the left anterior descending coronary artery from the pulmonary artery (ALADPA) is extremely rare. Here, we report a case of ALADPA in an adult that was treated with coronary artery bypass grafting using the left internal thoracic artery.

Sign in / Sign up

Export Citation Format

Share Document