CASE 13833 Published on 29.01.2018

Haematemesis in a patient with Tetralogy of Fallot

Section

Cardiovascular

Case Type

Clinical Cases

Authors

Veronica Attard, Luise Reichmuth

Mater Dei Hospital,Medical Imaging Department; Triq Dun Karm MSD 2090 Msida; Email:veronica2687@gmail.com
Patient

52 years, female

Categories
Area of Interest Lung, Cardiac ; Imaging Technique CT, Conventional radiography
Clinical History
A 52-year-old female patient with a history of palliated Tetralogy of Fallot (TOF) with pulmonary atresia, venticular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCAs) presented with haematemesis. The patient was transfused. An oesophagogastroduodenoscopy showed blood in the stomach without mucosal ulceration or active bleeding.
Imaging Findings
A chest radiograph demonstrated cardiomegaly and bilateral middle and lower lung zone airspace opacification. Contrast-enhanced computed tomography (CT) of the thorax, showed a VSD with an overriding aorta and dilatation of the aortic root, ascending aorta and aortic arch. Numerous collateral arteries were demonstrated at the base of neck, mediastinum and within the upper abdomen. No active bleeding was identified. There was collapse of the lingula, apical segment of left lower lobe and part of the right middle lobe. Diffuse ground-glass opacification was noted in the right lung, most pronounced in the right middle lobe and the basal segments of the lower lobes. The hepatic veins and inferior vena cava were dilated. There was mottled parenchymal contrast enhancement of the liver, in keeping with congestive heart failure. The remainder of the imaged upper abdominal organs were unremarkable.
Discussion
MAPCAs develop early throughout life and represent fetal primitive intersegmetal arteries [1]. Later on, these involute as the normal pulmonary arteries form. In certain conditions, when the pulmonary circulation is underdeveloped, MAPCAs become the main supply of blood to the lungs and connect the pulmonary arteries with the systemic circulation [2]. MAPCAs therefore act as alternatives to the systemic pulmonary arterial supply [3].

MAPCAs arise from the descending aorta (70%), branches of the aortic arch (15-20%) and the ascending aorta (10-15%) [2]. MAPCAs anastomose with the intrapulmonary arteries characteristically at or near the pulmonary hilum [1].

35–40% of patients with TOF and pulmonary atresia have MAPCAs [2].

TOF is the most common cyanotic congenital heart disease (CHD) in children, representing 5% to 7% of all CHD [3]. Moreover, it is also the most common complex CHD in adulthood.

The classic findings in TOF are:
1. overriding aorta
2. right ventricular hypertrophy
3. VSD
4. right ventricular outflow obstruction.

Patients often have underdeveloped or maldeveloped central pulmonary arteries.

In patients with TOF and marked infundibular stenosis, the pulmonary blood flow is markedly reduced and not functionally significant. This subset of TOF has been termed TOF with pulmonary atresia and constitutes a specific type of pulmonary atresia with VSD (PA-VSD) [1]. MAPCAs are most commonly demonstrated in PA-VSD (so-called severe TOF) [4]. PA-VSD is classified into three entities. In type A, the native pulmonary arteries are developed and the patent ductus arteriosus is the main supply. In type B (this case), both native pulmonary arteries and MAPCAs provide the main pulmonary supply. In type C, native pulmonary arteries are absent and the blood supply is only through MAPCAs [1].

This lady had a left Blalock shunt many years ago and later on a right internal mammary to pulmonary artery anastomosis. Cardiac MRI had confirmed that the pulmonary blood supply was via a multitude of aortopulmonary collaterals and none of the previously created shunts were patent.

Haemoptysis can result from erosion of collaterals into the respiratory tract. In the absence of signs of infection, the ground-glass changes demonstrated in this case, most likely represent pulmonary haemorrhage. Subsequent swallowing of blood might have lead to the presentation of haematemesis. Aspiration in the context of large volume haematemesis is another cause which could account for the pulmonary findings. Other complications of MAPCAs include pressure symptoms, on account of their size. The patient was treated conservatively, remains cyanotic and has not sustained further episodes of haematemesis.
Differential Diagnosis List
Rupture of MAPCAs, pulmonary haemorrhage, subsequent haematemesis
Rupture of major aortopulmonary collaterals into the airways
Subsequent swallowing of blood and resultant haematemesis
Pulmonary aspiration from bleeding collaterals in the abdomen
Final Diagnosis
Rupture of MAPCAs, pulmonary haemorrhage, subsequent haematemesis
Case information
URL: https://eurorad.org/case/13833
DOI: 10.1594/EURORAD/CASE.13833
ISSN: 1563-4086
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