Bastian was born 25.06.2013 suffering from a double inlet left ventricle (DILV). He had successfully undergone a Norwood-I procedure, aortic isthmus correction, and Glenn procedure at the Paediatric Hospital of the University of Erlangen, Germany without any significant complications.
However, following the fontan completion procedure on 29.11.2016, in which a pacemaker was implanted, Bastian developed a chylothorax within days of the operation. During the course of two intracardiac catheterisations to optimise haemodynamic performance, Bastian was proscribed with medication to lower his pulmonary pressure, placed on a three-month medium-chain triglyceride (MCT) diet and supporting preventative nutrition (due to his substantial weight loss). Unfortunately, none of these treatments were successful. During this the pacemaker didn´t work probably, requiring surgery to install a new unit.
After six months of having pleural drainages yet still suffering from a chylothorax, Bastian had to have a new catheter installed, which made fontan fenestration impossible. This procedure took place on 09.06.2017, replacing the pacemaker to also make it MRI compatible. Although Bastian was released from the ICU one day after the operation, he was in a significantly weakened physical condition.
Despite this operation, he continued to require the pleural drainage. Based on his depressed mood and weight the MTC diet was not continued. Fortunately, the right and left drainages lines were removed on 10.07.2017 and 21.07.2017, respectively. Between the removal of these drainages a cortisone treatment was also administered. He was released on 22.07.2017 with an effusion of approximately 1cm on each side.
Things were running well at home. Bastian was feeling well and showing visible signs of improvement. He was enjoying eating again and was able to return to preschool in September. However, in mid September he developed a cold and shortly after had a substantial pseudomonas Krupp attack with the cold remaining. Pleural effusion periodically increased up to 3cm during this time, however dropped again to 1.5cm. At the start of December, strong coughing symptoms were apparent and the increasing rate of effusion most likely triggered by an infection. Bastian’s oxygen saturation became apparent and he developed a lack of energy and low appetite. He was proscribed a therapy of Salbutamol and Budesonide inhalation. Unfortunately on 24.12.2017 Bastian began vomiting at night, developed fever and suffered significant breathlessness, with blood oxygen saturation falling towards 80 percent.
Our paediatric cardiologist observed effusion between 4 and 5cm on 27.12.2017 and we drove to the clinic in Erlangen on the same day. In the first attempt, Lasix (IV) was administered, which sadly did not have any considerable effect and required puncturing of 600ml of effusion. After the fitting of new pleural drainage tubes a further 800ml was drained. Oxygen saturation normalised again after this intervention. The coughing and presumably the infection declined. The effusion rate, which had been 70ml/24h and 170ml/24h for the right and left sides, respectively, increased over the course of a few weeks to 500ml/24h, a rate higher than any previously measured.
A professor from the clinic in Erlangen made it plain to us in a sobering discussion that, despite our hopes, the effusion alone is not triggering the infection, but rather that he interprets Bastian’s immune system as being too weak, given that an infection can lead to such symptoms. He told us that he does not believe the effusion can stop by themselves, giving Bastian a very bad prognosis. An MRI of the lymph system did not reveal major irregularities. In their final diagnosis, the medical team in Erlangen indicated complications to Bastian’s lymph system, referring him to a specialist clinic in Philadelphia, Pennsylvania (USA) as his last chance.