Everlasting Fifth Bow? This kind of heart disease is a little weird

 On November 2, 2016, Haohao (pseudonym), who was only 7 months old, developed cough and runny nose after vaccination. His parents took him to the Capital Institute of Pediatrics for treatment. Two days later, Hao Hao developed irritability, labored breathing, nasal fan, and three depressions (referring to depressions in the suprasternum fossa, intercostal space, subcostal and xiphoid process during inhalation). After a series of examinations such as echocardiography, it was found that he had an extremely rare congenital heart disease-the permanent fifth bow. In view of the critical condition, the green channel was opened immediately after the cardiac surgery consultation and Haohao was admitted directly to the heart disease. Intensive care unit (CICU) treatment.

Haohao's left ventricle is enlarged in a spherical shape, with a left ventricular end diastolic diameter of 43 mm, which is equivalent to the size of an adult. Due to the severe fifth arch constriction, as soon as Haohao was born, the left ventricular afterload increased sharply, resulting in severe damage to the left ventricular function. After careful treatment by CICU doctors, Haohao's respiratory infection was controlled and his heart function improved.

On the morning of November 17, 2016, Director Zhang of Cardiac Surgery performed the operation for Hao Hao. He chose to use the surgical plan of general anesthesia, deep hypothermia, lower body circulatory arrest, and selective cerebral perfusion. During the operation, the body temperature of the child should be lowered to below 25 degrees Celsius, and the blood circulation will be completely stopped in the lower body, with only a relatively small amount of blood flow to maintain cerebral perfusion. The key to surgery is to control the time to stop the circulation as much as possible within 30 minutes. That is to say, it is necessary to complete the resection and fine anastomosis of the narrowed blood vessels in only 30 minutes to minimize the damage caused by stopping the circulation. . After 5 hours of continuous fighting by medical staff, Haohao's operation was very successful. Haohao was in a stable condition after the operation. The tracheal intubation was removed on the second day and he recovered and was discharged from the hospital.

What is the "Eternal Fifth Bow"

Perpetual fifth bow is a very rare congenital heart disease. Since Van Praagh first described this type of malformation in 1969, only a few cases and a small number of cases have been reported worldwide. The Shanghai Children's Medical Center in China has reported 5 cases of surgery, but 2 cases died from the operation, with a mortality rate of 40%.

What is the performance of "Eternal Fifth Bow"

The permanent fifth arch is usually associated with other cardiovascular malformations, including interruption of the aortic arch, pulmonary atresia, patent ductus arteriosus, tetralogy of Fallot, and transposition of the great arteries.

If the fifth bow is simply perpetuated, hemodynamic changes are not significant, and there are often no obvious symptoms. If combined with coarctation of the aorta, it can increase the left ventricular afterload, reduce blood supply to the lower body, and cause high blood pressure to have pulse pressure differences between the upper and lower extremities, which will eventually lead to congestive heart failure. If combined with patent ductus arteriosus, it can lead to increased pulmonary circulation blood volume, increased left ventricular preload, left ventricular hypertrophy, and then pulmonary hypertension and right heart failure. In clinical examination, the disease can be detected by echocardiography, but due to the limitation of examination technology and physician experience, there is still a certain rate of misdiagnosis and missed diagnosis. Therefore, enhanced pulmonary angiography (CTA) or nuclear magnetic resonance (MRI) is the preferred method of examination.

How to treat the "Eternal Fifth Bow"

Since the permanent fifth arch generally coexists with the fourth arch on the same side, the two blood vessels are mostly on the same plane and will not form a vascular ring that affects the airway and esophagus. Therefore, if the permanent fifth bow has no stenosis, it usually does not need to be treated. If constriction is combined and it has a significant impact on heart function, surgical correction is required. Medical treatment methods include: interventional balloon expansion or stent graft expansion of vascular stenosis; surgical treatment methods include: artificial blood vessel replacement, patch widening, stenosis resection, and anterior wall pericardial patch expansion and reconstruction. Among them, resection and reconstruction can reduce the incidence of long-term restenosis to a certain extent.