His story begins with a long and difficult journey. EH, 13 months old, seriously ill Palestinian girl, arrived in Italy on 11 March 2024 as part of the humanitarian mission coordinated by the Prime Minister – final destination Gaslini Institute of Genoa – with an Air Force flight from Egypt, assisted by a team specialized in the transport of critical patients in difficult environments of the complex operational unit (Uoc) of neonatal and pediatric intensive care of the Ligurian hospital, composed of the doctor Andrea Moscatelli and the pediatric nurse Morgana Bacherini. The little girl was suffering from a complex and very serious heart disease and obstructive hydrocephalus. At Gaslini they rebuilt her ailing heart and subjected her to a series of life-saving operations that put her out of danger.
“Today she is discharged in excellent clinical conditions – says the medical director of Gaslini, Raffaele Spiazzi – She will have to follow a cardiological, cardiac surgical and neurosurgical follow-up but the probability of survival and long-term quality of life are favourable. These results, in cases like this complex, are only possible thanks to the great work of a multi-professional team that includes extremely trained doctors, nurses and perfusion technicians dedicated to treating the most complicated situations”. Her pathology was an extreme form of tetralogy of Fallot with pulmonary atresia, absence of the left pulmonary artery, and a single stenotized right pulmonary artery kept patent by a stent. Furthermore, she presented with obstructive tetraventricular hydrocephalus, in suspected Dandy Walker anomaly (the child had undergone a palliative surgical procedure at 2 weeks of age in Israel with stent placement). This is technically the picture illustrated by the doctors.
The little Palestinian girl was identified during the humanitarian mission and her transfer to Gaslini from Egypt, where she was with her mother, was organised. It was a transport “particularly at risk, since the child was in great pain due to the poor functionality of the pulmonary circulation – explains Moscatelli, director of the Gaslini Neonatal and Pediatric Intensive Care Unit – The cyanotic child survived only thanks to the perfusion of a single lung, with a transcutaneous oxygen saturation of less than 70%. Cyanosis is further aggravated by flying at high altitude, even in pressurized aircraft, as the partial pressure of oxygen is significantly reduced”.
The multidisciplinary management took place “through the coordinated action of 6 complex operational units of the institute: Intensive Care, Anesthesia, Radiology, Cardiology, Neurosurgery and Cardiac Surgery” explains Spiazzi. On March 19 in the hemodynamics room, the cardiology team led by Roberto Formigari performed a delicate diagnostic confirmation and dilation of the stent to improve the flow to EH’s right lung: the arterial saturation improved and his clinical condition stabilized, but it was clear that cardiac surgery would be necessary.
We opted for the correction of the heart disease, with the unknown of a poorly visualized left pulmonary artery. On April 3, cardiac surgeon Guido Michielon, director of the Gaslini Cardiac Surgery Unit, and his team performed a radical corrective operation. To create the connection between the heart and lungs, a valve from a human donor was needed, which was identified in Barcelona and transported to Genoa. This new valved pulmonary artery was connected between the right heart and the newly reconstructed pulmonary bifurcation, thus stabilizing heart function. “In extracorporeal circulation, the child’s left pulmonary artery was identified in the left lung” and a normal pulmonary bifurcation could be reconstructed. The left ventricle was tunneled to the aorta, separating the right and left parts of the heart.
After reconnection of the left pulmonary artery to the right pulmonary artery with reconstruction of the pulmonary bifurcation, this new pulmonary artery, equipped with a valve, is connected between the right heart and the pulmonary bifurcation. “The child leaves the operating room with a 4-chamber heart, separation between oxygenated and non-oxygenated blood, symmetrical pulmonary flow and on two lungs, 100% arterial saturation, without residual defects”, explains Michielon. From there she is transferred to intensive care where she is cared for post-operatively and prepared for the subsequent neurosurgical intervention. After MRI, the Neurosurgery team under the guidance of Gianluca Piatelli successfully performs neuroendoscopic treatment of hydrocephalus. All diagnostic and therapeutic procedures were well tolerated thanks to the work of the anesthesiology team, led by Andrea Wolfler, director of Anesthesiology, acute and procedural pain therapy, and the highly specialized staff of the neurosurgical operating room: the successful outcome of the procedure lays the foundation for good psychomotor development of the little patient.
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