CONGENITAL – Original Submission|Articles in Press

Early Experience With Reverse Double Switch Operation for the Borderline Left Heart

Published:September 27, 2022DOI:
      This study reviews our early experience with the “reverse” double switch operation (R-DSO) for borderline left hearts. A retrospective review of children with borderline left hearts who underwent R-DSO between 2017 and 2021 was conducted. Patient characteristics and early hemodynamic and clinical outcomes were collected. R-DSO was performed in 8 patients with no operative or postoperative deaths; 5 underwent decompressing bidirectional Glenn. Left ventricular (LV) poor-compliance was the dominant pathophysiology. Four patients had undergone staged LV recruitment but were not candidates for anatomical biventricular circulation due to LV hypoplasia and/or diastolic dysfunction. 7/8 patients had risk factors for Fontan circulation including pulmonary vein stenosis, pulmonary hypertension, and pulmonary artery stenosis. Median age at R-DSO was 3.7 years (19 months-12 years). All patients were in sinus rhythm at discharge. At median follow-up of 15 months (57 days-4.1 years) no mortalities, reoperations or heart transplants had occurred. All patients had normal morphologic LV systolic function. In one patient, pre-existing pulmonary hypertension (HTN) resolved after R-DSO. Reinterventions included transcatheter mitral valve replacement for residual mitral stenosis and neo-pulmonary balloon valvuloplasty. In 4 patients follow-up catheterization done at a median of 519 days (320 days-4 years) demonstrated median cardiac index of 3.2 L/min/m2 (2.2-4); median sub-pulmonary left ventricular end diastolic pressure was 9 mm Hg (7-15); median inferior vena cava/baffle pressure was 8 mm Hg (7-13). R-DSO is an alternative to anatomical biventricular repair or single ventricle palliation in patients with borderline left hearts and can result in low inferior vena cava pressures and favorable early results. This approach can also relieve pulmonary HTN and allow future transplant candidacy.

      Graphical Abstract



      R-DSO (reverse double switch operation), LV (left ventricle), mLV (morphologic left ventricle), mRV (morphologic right ventricle), BDG (bidirectional Glenn), HLHS (hypoplastic left heart syndrome), MS (mitral stenosis), AS (aortic stenosis), DORV (double outlet right ventricle), TGA (transposition of the great arteries), LV-PA (left ventricle to pulmonary artery), iEDV (end diastolic volume index), EF (ejection fraction), EFE (endocardial fibroelastosis), CI (cardiac index), LVEDP (left ventricular end diastolic pressure), RVEDP (right ventricular end diastolic pressure), LVEDV (left ventricular end diastolic volume), SVC (superior vena cava), IVC (inferior vena cava), ASD (atrial septal defect), PVR (pulmonary vascular resistance), PLE (protein losing enteropathy), ccTGA (congenitally corrected transposition of the great arteries), LVOT (left ventricular outflow tract), NYHA (New York Heart Association)
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      • Commentary: How far will we go?
        Seminars in Thoracic and Cardiovascular Surgery
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          The management of hypoplastic left heart syndrome (HLHS) has improved substantially over the past few decades; yet long-term outcomes are sub-optimal. Transplant-free survival at 6 years is around 60%.1 Complications of the Fontan circulation continue to plague late outcomes in this and other single ventricle patient populations.2 This has led to a renewed attempt to establish biventricular circulation3 or alternatively, identify transplant candidates early to improve overall outcomes. Prior studies indicate some success in recruiting the hypoplastic left ventricle (LV) for eventual incorporation as the systemic ventricle.
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