This is a case of a 12 year old girl who was diagnosed at birth with a double outlet right ventricle, pulmonary atresia, and interrupted inferior vena cava (IVC), with suspected Heterotaxy syndrome. A modified Blalock-Taussig shunt was done. She remained cyanotic due to a hypoplastic pulmonary artery bed and was considered unsuitable for further surgical intervention and was followed up at the Paediatric Cardiology clinic. At follow up at the age of 5 years old, cardiac catheterization to assess pulmonary arteries for possible surgery was conducted and she was incidentally found to have a dilated non-obstructed left renal pelvis. She was also found to be hypertensive and was treated with an angiotensin converting-enzyme inhibitor and a calcium channel blocker. Other treatment given for her cardiac disease included furosemide, digoxin, ferrous gluconate, and potassium supplementation. She developed progressive swelling and pain of her large joints from the age of 11 years old, and immune-mediated and destructive arthropathies were excluded. She was treated with a non-steroidal anti-inflammatory drug (NSAID). The swelling and pain of her large joints did not improve and radiologic investigations were found to be in keeping with secondary hypertrophic osteoarthropathy.In our patient, surgery was not possible for her heart condition and only conservative symptomatic treatment was offered. Her treatment remained a therapeutic challenge in view of the poly-pharmacy required to treat her multi-systemic condition. The side effects of these drugs are particularly undesirable in our patient, but had to be weighed against the poor quality of life she would have had if her treatment was focussed on her heart condition only.