Acquired and progressive coronary arterial fistulae in patients with single-ventricle physiology and treated with pulmonary vasodilators

Background: Cardiac dysfunction, arrhythmia, and hepatic fibrosis are well-known complications after right heart bypass surgery in patients with single-ventricle physiology. However, little is known about coronary arterial fistulae, and only a few reports have been published. This study aimed to elucidate the clinical characteristics of these rare coronary arterial fistulae that developed as complications in cases of single-ventricle physiology after right heart bypass surgery. Methods: We retrospectively investigated the clinical features and courses of patients who developed acquired and progressive coronary arterial fistulae after right heart bypass surgery in our hospital. Results: We identified three cases of coronary arterial fistulae out of 21 patients who underwent right heart bypass surgery. All three cases underwent cardiac catheterisation for post-operative evaluation and were administered pulmonary vasodilators of phosphodiesterase type V inhibitors, antiplatelet, anticoagulation, and diuretics. Moreover, they had common clinical features such as right-dominant single ventricle and long-term exposure to chronic hypoxia. Serial angiograms revealed acquired and progressive coronary arterial fistulae. In addition, coronary arterial fistulae contributed to their symptoms of heart failure. Conclusion: Patients with chronic hypoxia and dominant right ventricle, who are treated with phosphodiesterase type V inhibitors, should be followed up after right heart bypass surgery to monitor the possible development of coronary arterial fistulae. Moreover, the indication for pulmonary vasodilators in single-ventricle physiology after right heart bypass surgery should be optimised to avoid adverse effects.

A Tribute to the Pioneers of Right Heart Bypass: An Historical Review

Cavopulmonary anastomosis was first described by Carlon, Mondini, De Marchi in a canine model in 1951 and later, in the clinical practice, by Glenn in 1958. Total right heart bypass was first introduced by Fontan and Kreutzer in 1971, in each instance as treatment for tricuspid atresia. Several modifications of such a procedure followed the initial concept of the right atrium as a pumping chamber, including modifications aimed to minimize energy loss at the anastomotic level and arrhythmias. Tribute is given to our pioneers who developed such an operation aimed to treat any child with functionally univentricular hearts.

Development of cardiopulmonary bypass: A historical review

The idea of isolated organ perfusion, a precursor of cardiopulmonary bypass, came by Legalois in 1812. First isolated organ perfusion was described by Loebell in 1849. The first closed system for oxygenation and returning the blood through arteries was created by Frey and Gruber in 1885. Gibbon Jr. is considered the father of extracorporeal circulation. In spring of 1934 he began constructing a machine for extracorporeal circulation in Boston. He published the first description of this system in 1937. Gibbon won the grant of the International Business Machines Corporation for developing the machine in 1947. Together they developed Model I in 1949 and Model II in 1951. After a few unsuccessful attempts in 1952, the first successful surgical intervention on the heart (closure of atrial septal defect) using cardiopulmonary bypass was performed on May 6, 1953. In 1945, Kirklin and his working group reported on a series of eight successfully treated patients in a row who underwent surgery with extracorporeal circulation. First successful valve surgery under the direct vision was performed by Dodrill in 1952, using his ?Michigan Heart? machine as a right heart bypass. Using cardiopulmonary bypass, cardiac surgeons can deal with the complex cardiac pathology and save millions of lives.