Dr. Norberto Berber & Dr. Ignacio Lugones
Hospital General de Niños Pedro de Elizalde - Buenos Aires, Argentina
Clinical cases
This report will analyse two highly complex and very similar cases of two paediatric patients with congenital heart disease.
Case report 1: Female patient aged 18 months and weighing 9kg with a diagnosis of pulmonary atresia plus ventricular septal defect, plus systemic pulmonary collateral arteries (MAPCAS) with absence of pulmonary branches.
1. Cardiac catheterisation and evaluation echocardiography
With clinical suspicion of congenital heart disease, an echocardiogram is performed at 2 months of age. With the echocardiographic diagnosis, it was decided to continue with cardiac catheterisation.
Conclusions:
◾ Large, non-restrictive ventricular septal defect with bidirectional shunt.
◾ Small foramen ovale with left-to-right shunt.
◾ Pulmonary atresia with VSD and major aorto-pulmonary collateral. The right lung has unifocal circulation and the left lung has multifocal circulation. They are very hypoplastic.
◾ Same pressure in aorta, right and left ventricle.
2. Surgery
At 4 months of age, she was taken to surgery with the intention of performing a systemic-pulmonary anastomosis that could not be performed due to the absence of the pulmonary trunk and its right and left branches. Closure and subsequent evaluation was therefore decided.
The patient was monitored by the cardiology service with strict control of oxygen saturation.
3. Computed tomography angiography
At nine months, a CT angiography was performed to assess the systemic-pulmonary collaterals and evaluate therapeutic possibilities: stent implantation or unifocalisation surgery.
4. Next steps
Currently, after re-evaluation of the patient after several months without visits due to the pandemic, decreased oxygen saturation levels were recorded (80% average).
The Cardiology and Cardiovascular Surgery Departments decided in a meeting the possible scenarios for the resolution of the case.
Case report 2: 6-month-old male patient diagnosed with pulmonary atresia with ventricular septal defect and aorto-pulmonary collateral arteries (MAPCAS).
1. Cardiac catheterisation and X-ray images
Detection of pulmonary atresia with VSD, multifocal pulmonary circulation whose collaterals arise from the descending aorta and cardiomegaly.
2. Hospitalisation
Successive hospitalisations of the patient for respiratory problems.
3. Computed tomography angiography
◾ Measurement of the diameters of the collateral arteries and their location in space.
◾ Large subaortic VSD with aortic valve riding the septal defect.
◾ Detection of left aortic arch with incorrect outflows from the neck vessels .
4. Next step
Currently, after the diagnostic findings, the Cardiology and Cardiovascular Surgery service decide in athenaeum the possible scenarios for the resolution of the case.
Analysis of surgical possibilities and decisions
In order to thoroughly analyse the cases presented, they were discussed in an athenaeum with the participation of multiple specialists in the field.
The criticality of these cases implies a greater effort when it comes to making decisions and evaluating the different possibilities that patients have. Therefore, in order to have a better understanding of the complexity of the patients' anatomy and to be able to assess the feasibility of the possibilities proposed, three-dimensional reconstructions of each patient were requested, as well as a high-resolution 3D resin impression of case 1.
In case 1: The patient is currently under pharmacological treatment and is clinically stable, with a holistic murmur and peripheral pulses present and symmetrical.
Case 2: The patient is currently clinically stable with a continuous base murmur radiating to 4 foci and no cardiological treatment.
For these patients the scenarios analysed are as follows:
- A first scenario considers a surgical intervention. This type of corrective procedure would seem beneficial in principle, but one cannot ignore the fact that the patient would again be subjected to a thoracic opening, as well as involving not one but several successive interventions considering the risk involved, the probabilities of survival and the stress for the patient and her family.
- A second scenario is not to perform surgery but to promote the maintenance of the patient's health through palliative care until the patient demands it.
Given the delicate situation faced, it is also of utmost importance to be able to reassure families about the options that can be chosen and the possible scenarios once the decision has been made. 3D technologies also play an important role here, as they help to explain each situation more clearly.
"In the presence of complex cardiovascular pathologies that are difficult to resolve, 3D technologies provide the treating team with clarity in relation to the needs of each patient, enabling them to achieve diagnostic precision and to predict alternatives in advance. "
"We believe that the routine application of this type of technology is necessary to improve the quality of treatment for each individual patient, which would increase the probability of success in each case", says Dr. Norberto Berber.
For the time being, the possibilities have been evaluated in each of these patients, but given the complexity of the pathology and the probable complications in the short, medium and long term, the decision has not yet been taken and an informative meeting is awaited with the parents who will participate in this decision.
You may be interested in: "Tailor-made simulation in paediatric surgery: anomalous pulmonary venous drainage". Dr. Pedro Becker at the UC Christus Clinic (Chile), used 3D models to practice the corrective surgery on it and to be able to define the surgical approach.