Exercise testing and spirometry as predictors of mortality in congenital heart disease: Contrasting Fontan physiology with repaired tetralogy of Fallot

2018 ◽  
Vol 13 (6) ◽  
pp. 903-910 ◽  
Author(s):  
Keri M. Shafer ◽  
Alexander R. Opotowsky ◽  
Jonathan Rhodes
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Tetralogy Of Fallot ◽  
Exercise Testing ◽  
Congenital Heart ◽  
Predictors Of Mortality ◽  
Repaired Tetralogy Of Fallot ◽  
Fontan Physiology

Congenital Heart Disease in Adults

2017 ◽  
Author(s):  
Susan E. Haynes ◽  
Heather L. Bartlett ◽  
David J Skorton ◽  
Luke J Lamers
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Tetralogy Of Fallot ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Vena Cava ◽  
Congenital Diseases ◽  
Septal Defects ◽  
Atrial Septal Defects ◽  
Repaired Tetralogy Of Fallot

With advances in medical and surgical care, an increasing number of children with congenital diseases of the heart and vasculature now survive to adulthood. The proportion of adults who are affected by congenital heart disease is expected to continue to increase. Thus, it is important for clinicians to be knowledgeable about the care of these patients. This review examines acyanotic disorders (shunts and valvular lesions), vascular anomalies, cyanotic disorders, and women’s health issues. Figures show the anatomy of atrial septal defects, transcatheter closure of atrial septal defects, an anatomic cross section showing the atrioventricular septum, the anatomic positions of ventricular septal defects, a computed tomography scan of aortic coarctation, angiogram of a persistent left superior vena cava draining into the right atrium, systemic artery-to-pulmonary artery shunts, magnetic resonance image of a patient with repaired tetralogy of Fallot and long-standing pulmonary valve insufficiency, treatment of pulmonary valve regurgitation with a transcatheter pulmonary valve, computed tomographic images of a patient with atrial switch palliation of transposition of the great arteries and multiple baffle obstructions, stages in the repair of functional single ventricles, and echocardiograms of a patient with Ebstein anomaly. Tables list recommendations for pulmonary valve replacement in repaired tetralogy of Fallot, conditions in which pregnancy is high risk, and cardiac indications for fetal echocardiography. This review contains 12 highly rendered figures, 4 tables, and 62 references.

Congenital Heart Disease in Adults

2017 ◽  
Author(s):  
Susan E. Haynes ◽  
Heather L. Bartlett ◽  
David J Skorton ◽  
Luke J Lamers
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Tetralogy Of Fallot ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Vena Cava ◽  
Congenital Diseases ◽  
Septal Defects ◽  
Atrial Septal Defects ◽  
Repaired Tetralogy Of Fallot

With advances in medical and surgical care, an increasing number of children with congenital diseases of the heart and vasculature now survive to adulthood. The proportion of adults who are affected by congenital heart disease is expected to continue to increase. Thus, it is important for clinicians to be knowledgeable about the care of these patients. This review examines acyanotic disorders (shunts and valvular lesions), vascular anomalies, cyanotic disorders, and women’s health issues. Figures show the anatomy of atrial septal defects, transcatheter closure of atrial septal defects, an anatomic cross section showing the atrioventricular septum, the anatomic positions of ventricular septal defects, a computed tomography scan of aortic coarctation, angiogram of a persistent left superior vena cava draining into the right atrium, systemic artery-to-pulmonary artery shunts, magnetic resonance image of a patient with repaired tetralogy of Fallot and long-standing pulmonary valve insufficiency, treatment of pulmonary valve regurgitation with a transcatheter pulmonary valve, computed tomographic images of a patient with atrial switch palliation of transposition of the great arteries and multiple baffle obstructions, stages in the repair of functional single ventricles, and echocardiograms of a patient with Ebstein anomaly. Tables list recommendations for pulmonary valve replacement in repaired tetralogy of Fallot, conditions in which pregnancy is high risk, and cardiac indications for fetal echocardiography. This review contains 12 highly rendered figures, 4 tables, and 62 references.

scholarly journals Cardiopulmonary Exercise Testing—A Valuable Tool, Not Gatekeeper When Referring Patients With Adult Congenital Heart Disease for Transplant Evaluation

2019 ◽  
Vol 10 (3) ◽  
pp. 286-291 ◽  
Author(s):  
Jonathan N. Menachem ◽  
Nosheen Reza ◽  
Jeremy A. Mazurek ◽  
Danielle Burstein ◽  
Edo Y. Birati ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Exercise Testing ◽  
Congenital Heart ◽  
Adult Congenital Heart Disease ◽  
Cardiopulmonary Exercise Testing ◽  
Peak Oxygen Consumption ◽  
Cardiopulmonary Exercise ◽  
Advanced Therapies ◽  
Adult Congenital

Introduction: Treatment of patients with adult congenital heart disease (ACHD) with advanced therapies including heart transplant (HT) is often delayed due to paucity of objective prognostic markers for the severity of heart failure (HF). While the utility of Cardiopulmonary Exercise Testing (CPET) in non-ACHD patients has been well-defined as it relates to prognosis, CPET for this purpose in ACHD is still under investigation. Methods: We performed a retrospective cohort study of 20 consecutive patients with ACHD who underwent HT between March 2010 and February 2016. Only 12 of 20 patients underwent CPET prior to transplantation. Demographics, standard measures of CPET interpretation, and 30-day and 1-year post transplantation outcomes were collected. Results: Patient Characteristics. Twenty patients with ACHD were transplanted at a median of 40 years of age (range: 23-57 years). Of the 12 patients who underwent CPET, 4 had undergone Fontan procedures, 4 had tetralogy of Fallot, 3 had d-transposition of the great arteries, and 1 had Ebstein anomaly. Thirty-day and one-year survival was 100%. All tests included in the analysis had a peak respiratory quotient _1.0. The median peak oxygen consumption per unit time (_VO2) for all diagnoses was 18.2 mL/kg/min (46% predicted), ranging from 12.2 to 22.6. Conclusion: There is a paucity of data to support best practices for patients with ACHD requiring transplantation. While it cannot be proven based on available data, it could be inferred that outcomes would have been worse or perhaps life sustaining options unavailable if providers delayed referral because of the lack of attainment of CPET-specific thresholds.

Endothelial function state following repair of cyanotic congenital heart diseases

2013 ◽  
Vol 25 (2) ◽  
pp. 222-227 ◽  
Author(s):  
Mohammad Reza Sabri ◽  
Hooman Daryoushi ◽  
Mojgan Gharipour
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Endothelial Function ◽  
Tetralogy Of Fallot ◽  
Congenital Heart ◽  
Cyanotic Congenital Heart Disease ◽  
Vascular Endothelial ◽  
Vascular Endothelial Function ◽  
Flow Mediated Dilatation ◽  
The Mean

AbstractBackgroundRepairing cyanotic congenital heart disease may be associated with preserving endothelial function. The present study aimed to evaluate vascular endothelial function in patients with repaired cyanotic congenital heart disease.MethodsIn a case–control study conducted in 2012 in Isfahan, Iran, 42 consecutive patients aged <35 years who had suffered from different types of cyanotic congenital heart disease and had undergone complete repair of their congenital heart defect were assessed in regard to their endothelial function state by measuring flow-mediated dilatation and other cardiac function indices. They were paired with 42 sex- and age-matched healthy controls.ResultsThe mean flow-mediated dilatation was lower in patients with repaired cyanotic congenital heart disease than in the controls [6.14±2.78 versus 8.16±1.49 respectively (p<0.001)]. Significant adverse correlations were found between flow-mediated dilatation, age, and body mass indexes, in those who underwent repair surgery. In addition, flow-mediated dilatation had a positive association with the shortening fraction, ejection fraction, and tricuspid annular plane systolic excursion value, and it was also inversely associated with carotid intima-media thickness and the myocardial performance index. The mean of the flow-mediated dilatation was significantly higher in the group with tetralogy of Fallot along with complete repair before the age of 2.5 years and also in those patients with total anomalous pulmonary venous connection or transposition of the great arteries repaired with an arterial switch operation before 6 months of age, compared with the other two subgroups. This includes patients with a tetralogy of Fallot defect repaired after 4 years of age and those with complex cyanotic congenital heart disease that was repaired after 2.5 years of age (mean age at repair 9±6.1 years).ConclusionEarly repair of a cyanotic defect can result in the protection of vascular endothelial function and prevent the occurrence of vascular accidents at an older age.

scholarly journals Cardiopulmonary exercise testing in congenital heart disease: equipment and test protocols

2009 ◽  
Vol 17 (9) ◽  
pp. 339-344 ◽  
Author(s):  
T. Takken ◽  
A. C. Blank ◽  
E. H. Hulzebos ◽  
M. van Brussel ◽  
W. G. Groen ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Exercise Testing ◽  
Congenital Heart ◽  
Cardiopulmonary Exercise Testing ◽  
Cardiopulmonary Exercise

Heart Rate Recovery Following Exercise Testing in Pediatric Patients with Acyanotic Repaired Congenital Heart Disease

2022 ◽  
Author(s):  
Natalie S. Shwaish ◽  
Lindsey Malloy-Walton ◽  
Keith Feldman ◽  
Kelli M. Teson ◽  
Jessica S. Watson ◽  
...  
Keyword(s):  
Heart Rate ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Exercise Testing ◽  
Congenital Heart ◽  
Pediatric Patients ◽  
Heart Rate Recovery

scholarly journals Pattern of congenital heart disease among children presenting to the Uganda Heart Institute, Mulago Hospital: a 7-year review

2020 ◽  
Vol 20 (2) ◽  
pp. 745-752 ◽  
Author(s):  
Judith Namuyonga ◽  
Sulaiman Lubega ◽  
Twalib Aliku ◽  
John Omagino ◽  
Craig Sable ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Tetralogy Of Fallot ◽  
Congenital Heart ◽  
Heart Defects ◽  
Truncus Arteriosus ◽  
Deletion Syndrome ◽  
Mulago Hospital ◽  
22Q11.2 Deletion ◽  
Heart Institute

Background: Congenital heart disease (CHD) is the most common congenital anomaly in children. Over half of the deaths due to CHD occur in the neonatal period. Most children with unrepaired complex heart lesions do not live to celebrate their first birthday. We describe the spectrum of congenital heart disease in Uganda. Methods: We retrospectively reviewed the data of children with CHD who presented to the Uganda Heart Institute (UHI), Mulago Hospital Complex from 2007 to 2014. Results: A total of 4621 children were seen at the UHI during the study period. Of these, 3526 (76.3%) had CHD; 1941(55%) were females. Isolated ventricular septal defect (VSD) was the most common CHD seen in 923 (27.2%) children followed by Patent ductus arteriosus (PDA) 760 (22%) and atrial septal defects (ASD) 332 (9.4%). Tetralogy of Fallot (TOF) and Truncus arteriosus were the most common cyanotic heart defects (7% and 5% respectively). Dysmorphic features were diagnosed in 185 children, of which 61 underwent genetic testing (Down syndrome=24, 22q11.2 deletion syndrome n=10). Children with confirmed 22q11.2 deletion had conotruncal abnormalities. Conclusion: Isolated VSD and Tetralogy of Fallot are the most common acyanotic and cyanotic congenital heart defects. We report an unusually high occurrence of Truncus arteriosus. Keywords: Congenital heart disease; children; Uganda.

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