Abstract
Introduction. Hemodynamic correction of complex congenital heart defects with hypoplasia or the absence of one of the ventricles of the heart by the Fontan method remains the operation of choice. After performing the operation, hemodynamics is characterized by an increase in blood pressure and a decrease in blood flow velocity throughout the entire venous system, which in a number of cases leads to the development of a complex of complications, which was called "the Fontan disease". To optimize hemodynamics and reduce the risk of complications, various modifications of the Fontan operation were proposed. In 1990, to use for the bypass of the right divisions an extracardiac conduit were proposed. The aim of the study is to study the spectrum of complications after the Fontan operation in the modification of the extracardiac conduit, the reasons for their development and the frequency of repeated hospitalizations.
Material and methods. 41 (29.3%) patients were studied after Fontan operation in the modification of the extracardiac conduit, which was repeated, due to the development of complications in the period from 1 month to 15 years after the operation. In the first 3 years after surgery, 33 (80.5%) patients were re-admitted; in the period from 4 to 6 years, 4 (9.7%) patients were admitted; 3 (7.3%) came in 7–9 years after the operation; and 1 (2.4) the patient entered 15 years after Fontan operation. 16 (39%) have been repeatedly reported. In 13 patients Fontan operation was performed with a single ventricle of the heart; in 8 – with tricuspid valve atresia; in 6 patients with right ventricular hypoplasia syndrome; in 14 with other complex congenital heart defects. In 35 (85.4%) patients before the operation of Fontan was created bidirectional cavopulmonary anastomosis.
Results. The main reason for re-hospitalization of patients after Fontan operation was circulatory failure – 36 (87.8%) patients. In 16 (39%) patients, the circulatory failure phenomenon was a consequence of the development of protein deficiency enteropathy. Of the other complications, rhythm disturbances were observed in 2 cases, in 1 – there were episodes of hemoptysis, in 1 – arterial hypoxemia and in 1 – repeated thromboembolism. 22 (53.7%) patients with complications had hemodynamically significant problems: conduit stenosis – in 3; large aortolateral collaterals – in 9; in 4 – stenosis of pulmonary arteries; recanalization of the pulmonary artery trunk at 15; in 1 case, an additional left-sided upper vena cava was drained into the coronary sinus; 1 – veno-venous fistulae and 5 patients had a combination of several types of hemodynamic disorders. Conservative therapy was performed in all patients with complications. In 22 patients with hemodynamic disorders, 36 endovascular operations were performed.
Conclusion. The main reason for repeated hospitalizations after Fontan operation in the modification of the extracardiac conduit was a circulatory failure of 36 (87.8%). Of these, 20 (55.6%) were the cause of decompensation, hemodynamically significant disorders. The combination of conservative therapy and endovascular operations in 73% of cases have a persistent positive result. Temporary improvement or resistance to treatment is noted in patients with protein deficiency enteropathy.
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About the authors
- Shvedunova Valentina Nikolaevna, Dr. Med. Sc., Professor, Head of Department, orcid.org/0000-0003-0022-5667;
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Zelenikin Mikhail Mikhailovich, Dr. Med. Sc., Professor, Head of the Department, orcid.org/0000-0002-1298-2940;
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Putyato Niyole Al’phonsovna, Cand. Med. Sc., Head of the Department;
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Pursanov Manolis Georgievich, Dr. Med. Sc., orcid.org/0000-0002-1421-1795;
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Astrakhantseva Tat'yana Olegovna, Dr. Med. Sc., Chief Researcher, orcid.org/0000-0002-8393-0275;
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Kovalev Dmitriy Viktorovich, Dr. Med. Sc., Cardiovascular Surgeon, orcid.org/0000-0002-4586-9258;
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Turdieva Niso Sayitkulovna, Cand. Med. Sc., Cardiovascular Surgeon, orcid.org/0000-0002-2735-2087;
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Mustafina Leysan Il’darovna, Cardiologist
