Impact of pregnancy on the systemic right ventricle after a Mustard operation for transposition of the great arteries

Abstract

Pregnancy after a Mustard operation is clinically well tolerated but carries a risk of RV dysfunction, which is sometimes irreversible.

Management of Pregnancy in Patients With Complex Congenital Heart Disease: A Scientific Statement for Healthcare Professionals From the American Heart Association

“…159 Women with leftsided obstructive lesions, D-transposition of the great arteries (D-TGA) with Mustard or Senning operation, congenitally corrected transposition of the great arteries (CC-TGA), Eisenmenger syndrome, or significant subaortic ventricular dysfunction are at risk of developing pulmonary edema. [5][6][7]78,114,157,[160][161][162][163][164][165] Women with repaired right ventricular (RV) outflow tract obstruction (eg, tetralogy of Fallot or pulmonary atresia) are at risk of developing right-sided heart failure. 78,166,167 Pregnancy is contraindicated in women with severe systemic ventricular dysfunction (left ventricular ejection fraction <30%, NYHA class III or IV).…”

Pregnancy-Related Obstetric and Cardiologic Problems in Women After Atrial Switch Operation for Transposition of the Great Arteries

“…Similar data were described by Guédès et al, who noted that 25% of the patients suffered RV deterioration, without full recovery in 75% of them. 3 Impairment of ventricular function is clinically perceivable through the deterioration in functional ability. In the current study functional deterioration occurred in 11.7% of pregnancies (20.6% of patients), most commonly a decrease of 1 FC.…”

“…This is much lower than in the Guédès et al study, in which increased regurgitation occurred in 50% of cases. 3 Obstructions of the systemic venous or pulmonary venous baffle can occur at the venoatrial anastomosis site or within the corresponding atrium, caused by the position of the suture line, the extent of the atrial excision, or patch size, form or material. In succession, fibrosis, endothelialization and shrinking can occur.…”

Cardiovascular Management in Pregnancy

“…There may be preexisting ventricular dysfunction, particularly in the case of single ventricle physiology or in women with an anatomic right ventricle functioning in the systemic circulation. [9][10][11][12][13] Arrhythmias are common in adults with CHD as a result of preexisting substrates such as cardiac chamber dilatation, pressure or volume overload leading to myocardial hypertrophy and fibrosis, surgical scars, trauma to the conduction tissues, and presence of patch tissue in the myocardium. Supraventricular arrhythmias are more common than ventricular arrhythmias in this patient population.…”

“…Deterioration of the systemic RV resulting in clinical heart failure is the major concern and may be irreversible. 11,12,29,50,52 Expert evaluation of these patients is essential before pregnancy to understand their risk for pregnancy and to manage them during pregnancy.…”