PubMed · 9246821
The source did not provide an abstract. Follow the original record for more information.
Explore connections, maps & timelines
E Atik. 1996. [Complex heart diseases. From concept to evolution].. https://pubmed.ncbi.nlm.nih.gov/9246821/
Cite the original work for its findings. Save a collection to share your selection of sources.
KEEP EXPLORING
Optimal initial palliation and a subsequent staged approach is mandatory for high-risk Fontan candidates. We describe the case of mitral atresia with severe tricuspid regurgitation and pulmonary hypertension successfully managed by repeated palliation from the neonatal period and 2-stage Fontan surgery. A 1-month-old boy diagnosed with mitral atresia and double-outlet right ventricle underwent pulmonary artery banding at 1 month of age, followed by repeated pulmonary artery banding accompanied by tricuspid annuloplasty and atrial septal defect enlargement at 6 months. Because of the presence of pulmonary artery distortion, right ventricular dysfunction, and borderline pulmonary vascular resistance, a hemi-Fontan procedure was conducted with extended pulmonary artery plasty when the boy was 3 years and 8 months old. Cardiac catheterization done 3 months after showed improvement in risk factors, and the final Fontan operation (total cavopulmonary connection) was successfully done in conjunction with repeated tricuspid annuloplasty when the boy was 4 years and 5 months old. The patient remains in excellent clinical condition at the last follow-up 5 years after the final Fontan procedure with sinus rhythm and good ventricular function.
We describe an alternative technique to the extracardiac Fontan procedure in a patient with a univentricular heart, in which the inferior caval vein and the hepatic vein drained separately into the common atrium and the intraatrial orifice of the hepatic vein was adjacent to the opening of the left lower pulmonary vein.
In the absence of a ventricular pump, the status of the pulmonary circulation is crucial to the success of the Fontan operation. In an updated version (1999) of the optimal criteria for the Fontan operation, several of these criteria address the pulmonary circulation: pulmonary artery pressure, pulmonary vascular resistance, pulmonary artery size, and absence of significant pulmonary artery branch stenosis. This chapter reviews the role of the pulmonary circulation in a successful Fontan operation, with a particular emphasis on surgical techniques to repair severe distal or hilar pulmonary artery stenosis or atresia. The special situation of the patient with a single pulmonary artery is also addressed. Severe hilar pulmonary artery stenosis or atresia can be repaired by the technique of intrapulmonary pulmonary artery reconstruction with pericardial patch or tube and allow the successful completion of the Fontan operation. In the selected patient with a single pulmonary artery and optimal hemodynamics, the Fontan operation is possible with good outcome. However, further experience is needed in a larger number of patients to assess the long-term outcome of these treatment strategies.