Only 4 cases in the world, the first in Italy: Ylenia Mellaro, optician, born 36 years ago in Rome with a heart without a ventricle, becomes the mother of twins. The exceptional event happened in Rome thanks to the determination of the young woman and the joint work of the specialists from the Bambino Gesù pediatric hospital – who have assisted her since she was a child due to her serious congenital heart disease – and the A. Gemelli Irccs university hospital, where the cesarean section was performed. Mother and children, a boy and a girl, Flavio and Giulia, are well: “I knew I was taking a lot of risks with my heart in half, but I wanted to become a mother. The birth of the twins was an indescribable emotion.”
What is univentricular heart
The twins’ mother was born with a heart missing the right ventricle, the portion of the heart muscle that pushes blood towards the lungs for oxygenation. It is a complex congenital heart disease that requires a series of corrective interventions within the first years of life. In this specific case, the Bambino Gesù patient underwent a single operation at the age of 4 to build an alternative circulation system, called Fontan, which brings blood from the vena cava directly to the lungs, without passing through the Heart. This condition subjects the organism to constant overwork: the circulation supported by a single ventricle requires, in fact, close therapies and controls throughout life; in 5% of cases a transplant is achieved.
A special team for “adult congenitals”
At Bambino Gesù there is a team of specialists who take care, for therapeutic continuity, of children with a heart disease who have come of age. Thanks to advances in pediatric cardiac surgery, adults born with heart disease (identified with the acronym ACHD, Adults with Congenital Heart Disease) are increasingly numerous today. Even though they have the disease under control, they cannot consider themselves completely cured. For this reason they require specific assistance.
As part of this activity, a multidisciplinary group of the two institutions called GUCH (Grown Up Congenital Heart) has long been present, which deals with the management of pregnancies of heart disease patients (through pre- and post-conceptional meetings and shared clinical-assistance documents), the birth planning (timing and methods) and post-pregnancy follow-up. The group includes the team from the Adult Congenital Cardiology Unit of Bambino Gesù, led by Gianfranco Buteratogether with the Fetal and Perinatal Medicine and Surgery team directed by Leonardo Caforio; for Gemini the group of cardiologists is coordinated by Massimo Massetti And Antonio Amodeo and the group of gynecologists from Antonio Lanzone.
Pregnancy and the birth of twins
In the world there are only 4 cases of twin births from mothers with a univentricular heart and Fontan circulation. In the case of such complex congenital heart diseases, in fact, pregnancy subjects the entire organism to greater stress and risks: blood volume and cardiac activity increase; organs such as the liver, kidneys and intestines can suffer. However, in the absence of other complications, according to the indications of the international protocols on the matter, it is possible to undertake the path of maternity. This is the case of the mother of the twins who, under strict medical supervision, completed a pregnancy without problems – the first case in Italy. The birth of the twins, both weighing 1.4 kg, took place via cesarean section at the Gemelli Polyclinic at the 34th week of pregnancy, a few days ahead of the expected due date. After just ten days of hospitalization, mother and children were able to return home.
The mother says: “I knew I was taking risks with my special heart, a half heart, but I am very determined and I really wanted to be a mother. In this adventure I was followed step by step by the doctors of Bambino Gesù and the Gemelli Polyclinic. Thanks to them I had a beautiful pregnancy and birth and the birth of my children was an indescribable emotion. I would like to tell women who experience the same situation as me not to be afraid and to rely on specialized centers to make their dream of motherhood come true “.