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The so-called "one-and-a-half" ventricular repair: where are we after 40 years?
Ujjwal Kumar Chowdhury1, Robert H Anderson2, Niraj Nirmal Pandey3
1Department of Cardiothoracic and Vascular Surgery, National Institute of Medical Sciences & Research, Jaipur, RJ, India.
Insights
The one-and-a-half ventricle repair offers an alternative to the Fontan circulation, with acceptable risks and good long-term outcomes. This surgical approach can reduce risks for biventricular repair and improve patient condition.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Surgery
- Cardiac Surgery Outcomes
Background:
- The "one-and-a-half ventricle repair" is an alternative surgical strategy for complex congenital heart disease, often considered when Fontan circulation or biventricular repair carries high risks.
- Indications, optimal timing, and long-term results of this procedure remain incompletely defined, necessitating further clarification.
Purpose of the Study:
- To elucidate the indications, surgical timing, and clinical outcomes associated with the "one-and-a-half ventricle repair."
- To compare its safety and efficacy against the Fontan circulation and high-risk biventricular repair strategies.
Main Methods:
- A comprehensive review of 201 investigations was conducted.
- Key aspects evaluated included patient selection, atrial septal fenestration, management of the azygos vein and pulmonary regurgitation, superior caval vein flow dynamics, subpulmonary ventricle function, and the role of superior cavopulmonary connections.
- Subsequent eligibility for biventricular repair conversion and long-term functional results were also assessed.
Main Results:
- Operative mortality varied from 3% to 20% depending on the surgical era.
- Complications included a 7% risk from pulsatile superior caval vein flow and up to one-third incidence of supraventricular arrhythmias.
- Actuarial survival reached 80-90% at 10 years, with two-thirds of patients in good condition after 20 years. Notably, no cases of plastic bronchitis, protein-losing enteropathy, or hepatic cirrhosis were reported.
Conclusions:
- The "one-and-a-half ventricle repair" can serve as a definitive palliative procedure with risks comparable to Fontan circulation conversion.
- This approach effectively lowers the surgical risk associated with biventricular repair and mitigates the "Fontan paradox."
- The procedure demonstrates favorable long-term survival and functional outcomes, offering a viable alternative for select pediatric cardiac patients.
Objectives:
The indications, timing, and results of the so-called "one-and-a-half ventricle repair", as a surgical alternative to the creation of the Fontan circulation, or high-risk biventricular repair, currently remain nebulous. We aimed to clarify these issues.
Methods:
We reviewed a total of 201 investigations, assessing selection of candidates, the need for atrial septal fenestration, the fate of an unligated azygos vein and free pulmonary regurgitation, the concerns regarding reverse pulsatile flow in the superior caval vein, the growth potential and function of the subpulmonary ventricle, and the role of the superior cavopulmonary connections as an interstage procedure prior to biventricular repair, or as a salvage procedure. We also assessed subsequent eligibility for conversion to biventricular repair and long-term functional results.
Results:
Reported operative mortalities ranged from 3% to 20%, depending on the era of surgical repair with 7% risk of complications due to a pulsatile superior caval vein, up to one-third incidence of supraventricular arrhythmias, and a small risk of anastomotic takedown of the superior cavopulmonary connection. Actuarial survival was between 80% and 90% at 10 years, with two-thirds of patients in good shape after 20 years. We found no reported instances of plastic bronchitis, protein-losing enteropathy, or hepatic cirrhosis.
Conclusions:
The so-called "one-and-a-half ventricular repair", which is better described as production of one-and-a-half circulations can be performed as a definitive palliative procedure with an acceptable risk similar to that of conversion to the Fontan circulation. The operation reduces the surgical risk for biventricular repair and reverses the Fontan paradox.
