Long-term outcome of patients with transposition of the great arteries and a systemic right ventricle: A systematic review and meta-analysis

Ramandi, Mohammad Mostafa Ansari; Yarmohammadi, Hossein; Gareb, Barzi; Voors, Adriaan A.; Melle, Joost P. van

doi:10.1016/j.ijcard.2023.131159

Cited by 2 publications

(7 citation statements)

References 63 publications

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“…Moreover, the incidence of mortality and hospitalization for heart failure was not associated with the average age at baseline. 202 The prognosis of patients with TGA has been investigated in several studies. One study reported that decreased left ventricular ejection fraction (LVEF) and right ventricular ejection fraction (RVEF), higher NT-proBNP levels, and a New York Heart Association (NYHA) class ≥ II are associated with a poor outcome in patients with TGA and SRV.…”

Section: Diagnosis and Treatment Of Tgamentioning

confidence: 99%

“…One study reported that decreased left ventricular ejection fraction (LVEF) and right ventricular ejection fraction (RVEF), higher NT-proBNP levels, and a New York Heart Association (NYHA) class ≥ II are associated with a poor outcome in patients with TGA and SRV. 202 Tricuspid regurgitation is frequent in survivors and predisposes them to progressive worsening, while sudden arrhythmia and heart failure are the most common reasons for late death. 199 Some patients may require placement of a ventricular assist device or cardiac transplant.…”

Section: Diagnosis and Treatment Of Tgamentioning

confidence: 99%

“…Patients with ccTGA also had an increased hospitalization for heart failure than those with TGA after atrial switch (4.3 vs. 3.2 per 100 patients/year); however, the rates of ventricular and supraventricular arrhythmias were not different between them. 202 A recent study reported that patients with ccTGA experience various stages of deterioration leading to end-stage heart failure or death over time. The incidence of death significantly increased between the ages of 51 and 69 years.…”

Section: Pathophysiology Of Congenitally Corrected Transposition Of T...mentioning

confidence: 99%

“…Therefore, ccTGA is often diagnosed later, which makes the SRV more susceptible to dysfunction, ultimately leading to heart failure. 202 Progressive decreases in SRV function and tricuspid regurgitation are the main complications of ccTGA. In a previous study, 34% of patients with ccTGA had reduced SRV function, and this percentage increased to 46% after 6.5 years of follow-up.…”

Section: Pathophysiology Of Congenitally Corrected Transposition Of T...mentioning

confidence: 99%

“…Patients with TGA and systemic right ventricle (SRV) have increased hospitalizations and mortality because of heart failure. Moreover, the incidence of mortality and hospitalization for heart failure was not associated with the average age at baseline 202 . The prognosis of patients with TGA has been investigated in several studies.…”

Section: Transposition Of the Great Arteriesmentioning

confidence: 99%

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Congenital heart disease: types, pathophysiology, diagnosis, and treatment options

Meng,

Song,

Zhang

et al. 2024

MedComm

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Congenital heart disease (CHD) is a structural abnormality of the heart and/or great vessels and patients with CHD are at an increased risks of various morbidities throughout their lives and reduced long‐term survival. Eventually, CHD may result in various complications including heart failure, arrhythmias, stroke, pneumonia, and sudden death. Unfortunately, the exact etiology and pathophysiology of some CHD remain unclear. Although the quality of life and prognosis of patients with CHD have significantly improved following technological advancement, the influence of CHD is lifelong, especially in patients with complicated CHD. Thus, the management of CHD remains a challenge due to its high prevalence. Finally, there are some disagreements on CHD among international guidelines. In this review, we provide an update of the pathophysiology, diagnosis, and treatment in most common type of CHD, including patent foramen ovale, atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus, coarctation of the aorta, transposition of the great arteries, congenitally corrected transposition of the great arteries, coronary anomalies, left and right ventricular outflow tract obstruction, tetralogy of Fallot and Ebstein anomaly. In particular, we focus on what is known and what is unknown in these areas, aiming to improve the current understanding of various types of CHD.

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Section: Diagnosis and Treatment Of Tgamentioning

confidence: 99%

Section: Diagnosis and Treatment Of Tgamentioning

confidence: 99%

Section: Pathophysiology Of Congenitally Corrected Transposition Of T...mentioning

confidence: 99%

Section: Pathophysiology Of Congenitally Corrected Transposition Of T...mentioning

confidence: 99%

Section: Transposition Of the Great Arteriesmentioning

confidence: 99%

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Congenital heart disease: types, pathophysiology, diagnosis, and treatment options

Meng,

Song,

Zhang

et al. 2024

MedComm

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Defining the causes for Fontan circulatory failure in total cavopulmonary connection patients

Van Puyvelde,

Rega,

Budts

et al. 2024

Interdisciplinary CardioVascular and Thoracic Surgery

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OBJECTIVES This study aims to identify causes of failure in Fontan patients with a total cavopulmonary connection. METHODS We conducted a comprehensive review of all patients who underwent a total cavopulmonary connection procedure at our centre between 1988 and 2023, aiming to identify and analyze the factors contributing to Fontan failure (defined as mortality, heart transplantation, Fontan takedown, protein-losing enteropathy, plastic bronchitis, or New York Heart Association Functional Classification class III or IV). RESULTS The study included 217 patients (median age at time of Fontan completion 3.7 years) with a median follow-up of 12.7 years (IQR, 7.2; 17.7). Systolic ventricular function decreased significantly over time in patients with right ventricular dominant morphology (P = 0.002), while systolic ventricular function remained stable in patients with left ventricular dominant morphology. Fontan failure occurred in 24 patients, with estimated freedom from Fontan failure rates of 97.7% (95% CI, 95–99) at 1 year, 93.9% (95% CI, 89–97) at 15 years and 77.2% (95% CI, 65–86) at 20 years follow-up. Systolic ventricular dysfunction was the most common cause of failure (29%), followed by atrioventricular valve regurgitation (16.7%), a high pulmonary vascular resistance (16.7%), restrictive pathophysiology (16.7%) and obstruction (12.5%). Patients with right ventricular dominance developed most often systolic ventricular dysfunction, while patients with left ventricular dominant morphology developed most often restrictive pathophysiology or a high pulmonary vascular resistance. CONCLUSIONS Approximately 10% of patients experienced Fontan failure within 15 years postoperatively. Patients with right ventricular dominance experienced progressive decline due to systolic dysfunction, while those with left ventricular dominance exhibited failure due to restrictive pathophysiology or high pulmonary vascular resistance.

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Long-term outcome of patients with transposition of the great arteries and a systemic right ventricle: A systematic review and meta-analysis

Cited by 2 publications

References 63 publications

Congenital heart disease: types, pathophysiology, diagnosis, and treatment options

Congenital heart disease: types, pathophysiology, diagnosis, and treatment options

Defining the causes for Fontan circulatory failure in total cavopulmonary connection patients

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