Thanks to the efforts of doctors at Almazov Centre, a woman with a severe and extremely rare comorbidity has found the long-awaited happiness of motherhood. The expectant mother knew about her congenital heart disease (large ventricular septal defect) since childhood. Even then, the patient noted breathing difficulty while lying down and during exertion. Her parents refused surgical repair of the heart defect in childhood, although it was offered several times. Because of this, over the years, she developed irreversible complications with severe pulmonary arterial hypertension and Eisenmenger syndrome.
At the age of 11, the girl had an appendectomy, which led to severe abdominal adhesions. The adhesions spread throughout the abdominal cavity and impeded the proper functioning of digestive organs. When the woman was 25 years old, doctors had to remove her uterine appendages on one side due to the ovarian neoplasm. Unfortunately, this procedure further exacerbated the adhesions. In 2017, the patient underwent a series of abdominal surgeries for adhesive intestinal obstruction with injury to the small bowel, small bowel stoma, attempted adhesiolysis and closure of the stoma.
The condition of the young woman required the use of PAH-specific therapy (special drugs for the treatment of pulmonary arterial hypertension), which was prescribed at her place of residence. Severe pulmonary arterial hypertension is regarded as an absolute contraindication for pregnancy, since it is associated with an extremely high risk of death for both the potential mother and the fetus.
However, despite all the warnings, at the age of 38, the woman firmly decided to become a mother. She unknowingly quitted PAH-specific therapy as she believed that taking drugs could harm the baby.
“Unfortunately, despite the recommendations of doctors to take PAH-specific drugs for life, many women cancel them on their own already at the stage of pregnancy planning, without consulting their doctor. Knowing that pregnancy is contraindicated, they hide it and refuse to be observed. They have a chance to carry and give birth to a baby without an unfavorable outcome for themselves only in close cooperation with specialists and with drug therapy,” says Olga Li, Deputy Medical Chief and obstetrician-gynecologist.
From the early stages of pregnancy, the patient received remote consultations by cardiologists and obstetricians-gynecologists of Almazov Centre and from the first visit she was assigned to the IV risk group (extremely high risk of maternal mortality according to the WHO scale). The woman categorically refused to terminate pregnancy for medical reasons, so resuming PAH-specific therapy was strongly recommended.
“Given the extremely high risk of cardiovascular complications during pregnancy, at 24 weeks, the woman was hospitalized to the Department of Complicated Pregnancy of the Perinatal Centre for additional examination and further management by a multidisciplinary team,” comments Tatyana Pervunina, Director of the Institute of Perinatology and Pediatrics.
The patient was admitted in an extremely severe condition, PAH-specific therapy was prescribed along with antiarrhythmic drugs.
The medical team including cardiologists, an internist, obstetrician-gynecologists, anesthesiologists, neonatologists, pediatric intensivists, a pulmonologist and a clinical pharmacologist repeatedly discussed the patient’s condition.
At 29 weeks, the pregnant woman felt worse, she had a heart rhythm disturbance, her oxygen saturation dropped and pulmonary hypertension progressed further. It led the doctors to decide on the early delivery by C-section. Given the history of adhesive intestinal obstruction, the surgery was performed jointly by obstetricians-gynecologists (O. Li and N. Osipova) and an abdominal surgeon (I. Danilov).
The work of anesthesiologists should be especially recognized. Anesthesia was administered with continuous invasive hemodynamic monitoring, similar to cardiac surgery, and constant inhalation of nitric oxide. General anesthesia with mechanical ventilation in patients with pulmonary arterial hypertension associated with congenital heart disease is considered the highest risk. Therefore, regional anesthesia is recognized as the method of choice for this condition. Together with cardiac anesthesiologists, a difficult decision was made: to start the surgery and delivery under epidural anesthesia, and should the duration of surgery increase, convert to combined anesthesia with mechanical ventilation. Considering that the surgery exceeded 6 hours and intraoperative blood loss was 1800 ml, the anesthetic plan was maintained.
A premature baby boy weighing 1460 g and measuring 39 cm, with an Apgar score of 6–7, received therapy for 20 days in the NICU of the Perinatal Centre, including respiratory support, partial parenteral nutrition. Then the baby was transferred to the ICU of the Neonatal Physiology Department. At a month and a half, he was transferred to a ward for a joint stay with his mother.
The mother received further treatment in cardiology department No. 9, where she was managed by cardiologist Taisiya Galanskaya.
Thanks to a comprehensive multidisciplinary approach and high-tech treatment methods, a team of like-minded professionals managed to save life of both mother and baby. After 1 month and 25 days after delivery, the mother and child were discharged for further follow-up at the place of residence.