Pulmonary outflow obstruction protects against heart failure in adults with congenitally corrected transposition of the great arteries

Helsen, Frederik; Meester, Pieter De; Keer, Jan Van; Gabriels, Charlien; Bruaene, Alexander Van De; Herijgers, Paul; Rega, Filip; Meyns, Bart; Gewillig, Marc; Troost, Els; Budts, Werner

doi:10.1016/j.ijcard.2015.05.142

Cited by 39 publications

(21 citation statements)

References 29 publications

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“…Some rare examples are in cases of ccTGA [5–8]. Pulmonary outflow obstruction in ccTGA not only protects the pulmonary arterial bed, but also protects the left-sided tricuspid valve and RV from failure [9]. In this situation, the morphologic LV has a trained effect as that with pulmonary artery banding; the increased left ventricular pressure compresses the septum, maintaining the tricuspid valve leaflet coaptation.…”

Section: Discussionmentioning

confidence: 99%

Hemodynamically balanced congenitally corrected transposition of the great arteries with a large ventricular septal defect, and subvalvular pulmonic stenosis: a case report

Cho

Lee

et al. 2019

J Med Case Reports

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Background Adults with unoperated congenitally corrected transposition of the great arteries are rare but form a distinct group among adults with congenital heart disease. Patients with congenitally corrected transposition of the great arteries often have one or more associated cardiac anomalies that dictate the need for, and timing of, surgical intervention in childhood. However, in a proportion of patients, the hemodynamics does not require surgical attention during childhood, and, in some patients, a correct diagnosis is not established until adulthood. Here we report an adult case of unoperated congenitally corrected transposition of the great arteries with a large ventricular septal defect and probable pulmonary arterial hypertension. Case presentation Our patient was a 46-year-old Korean man. Transthoracic echocardiography and cardiac catheterization demonstrated hemodynamically balanced ventricles with a non-regurgitant systemic atrioventricular valve, normal pulmonary arterial pressure, and a reasonable difference between the oxygen saturation values of the aorta and pulmonary trunk, even with the presence of a large ventricular septal defect. Further morphological assessments using cardiac computed tomography and three-dimensional modeling/printing of his heart revealed that the mitral valve was straddling over the posteriorly positioned ventricular septal defect, which could explain the functional and anatomical subvalvular pulmonary stenosis and a small amount of shunt flow through the large ventricular septal defect. We interpreted this combination of cardiac defects as able to sustain his stable cardiac function. Thus, we decided to maintain his unoperated status. Conclusion A detailed anatomical understanding based on transthoracic echocardiography, cardiac computed tomography, and three-dimensional printing can justify a decision to not operate in cases of congenitally corrected transposition of the great arteries with hemodynamically balanced pulmonary stenosis and a ventricular septal defect, as observed in the present case. Electronic supplementary material The online version of this article (10.1186/s13256-019-2145-1) contains supplementary material, which is available to authorized users.

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Section: Discussionmentioning

confidence: 99%

Hemodynamically balanced congenitally corrected transposition of the great arteries with a large ventricular septal defect, and subvalvular pulmonic stenosis: a case report

Cho

Lee

et al. 2019

J Med Case Reports

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“…Symptomatology of these patients depends on the criticality of the associated anomalies and right ventricular function [ 8 ]. These patients are known to develop congestive heart failure as the right ventricle is subjected to both pressure and volume overload [ 9 ]. Since pulmonary outflow obstruction is naturally protective against heart failure in CCTGA patients, pulmonary artery banding shows favorable results [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

“…These patients are known to develop congestive heart failure as the right ventricle is subjected to both pressure and volume overload [ 9 ]. Since pulmonary outflow obstruction is naturally protective against heart failure in CCTGA patients, pulmonary artery banding shows favorable results [ 9 ]. This is due to an increased afterload on the left ventricle, which leads to reduced interventricular septal shift to the LV, resulting in reduced deformation of RV shape and function [ 10 ].…”

Section: Discussionmentioning

confidence: 99%

Congenitally Corrected Transposition of the Great Arteries in a Septuagenarian from the Developing Country of Pakistan

Shahab

Ashiqali

Atiq

2018

Cureus

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Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital cardiac defect with atrioventricular and ventriculoarterial discordance which leads to heart failure and limits patients’ lifespan. We report the case of a 70-year-old lady, from a poor province in Pakistan, who presented for the first time with palpitations and was diagnosed to have CCTGA. She had moderate pulmonic valve stenosis which was protective against heart failure. She had six children all born via spontaneous vertex delivery in her local village. This case exemplifies the fact that pulmonic stenosis is favourable for patients with CCTGA. In a country where the average life expectancy of females is only 68 years, the survival of our patient with CCTGA beyond the average lifespan is indeed interesting.

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“…Even in the presence of dysplasia of the TV, with severe preoperative regurgitation, concomitant TV repair is rarely needed at the time of the anatomic correction. 2,3 On the other hand, in patients not suitable for anatomic correction, the native well-balanced pulmonary stenosis has a protective effect on TV and mRV function, 24 and thus preventive PAB with limited atrial septal communication might be useful as a long term or definitive palliation to mimic the favorable history of patients with ccTGA and limited LVOTO.…”

Section: Discussionmentioning

confidence: 99%

What surgical improvements are needed to prove that anatomic repair is superior to physiologic repair in the majority of patients with corrected transposition of the great arteries?

Hraška

Mitchell

Woods

et al. 2017

The Journal of Thoracic and Cardiovascular Surgery

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Pulmonary outflow obstruction protects against heart failure in adults with congenitally corrected transposition of the great arteries

Abstract: POTO is associated with an improved event-free survival in adults with ccTGA.

Cited by 39 publications

References 29 publications

Hemodynamically balanced congenitally corrected transposition of the great arteries with a large ventricular septal defect, and subvalvular pulmonic stenosis: a case report

Hemodynamically balanced congenitally corrected transposition of the great arteries with a large ventricular septal defect, and subvalvular pulmonic stenosis: a case report

Congenitally Corrected Transposition of the Great Arteries in a Septuagenarian from the Developing Country of Pakistan

What surgical improvements are needed to prove that anatomic repair is superior to physiologic repair in the majority of patients with corrected transposition of the great arteries?

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