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Revista Portuguesa de Medicina Geral e Familiar
Print version ISSN 2182-5173
Abstract
CASTRO, Carlos Rocha and FACHADO, Francisco. ARCAPA syndrome: the role of the family physician in the management of a rare disease. Rev Port Med Geral Fam [online]. 2023, vol.39, n.1, pp.67-72. Epub Mar 30, 2023. ISSN 2182-5173. https://doi.org/10.32385/rpmgf.v39i1.13470.
Introduction:
ARCAPA syndrome (anomalous right coronary artery from the pulmonary artery) is a rare congenital heart disease. Patients may present with acute myocardial infarction or sudden death, or remain asymptomatic, being diagnosed accidentally. The family physician (FP) is who can best understand the patient's perspective on the disease and integrate it into his biopsychosocial context.
Case description:
Male, 48 years old, high-intensity physical exercise practitioner. History of generalized anxiety disorder. In February 2019, due to a febrile episode and suspected pneumonia, a pulmonary CT scan was performed, which revealed “extensive pericardial vascular ectasias”, which prompted a referral to the cardiology service by the FP. CT angiography was later performed and confirmed “ARCAPA syndrome”. The patient recovered from pneumonia and remained asymptomatic. Given the risk of sudden death, surgery was proposed, and physical exercise was recommended to be stopped. Due to the absence of symptoms, the patient began to express incomprehension towards this accidental diagnosis, exacerbating his anxious pathology. Using a patient-centered approach, the FP sought to understand the patient’s expectations, feelings, and fears, as well as involve the family in every step of his decisions. The patient, on his own initiative, continued to exercise, stating that the suspension would aggravate his mental health. He also rejected the surgical proposal. Currently, he has regular follow-up appointments with the FP and the cardiologist for symptom surveillance.
Comment:
In addition to its rarity, this case reflects the importance of patient-centered medicine, especially after the diagnosis of a pathology with such an impact on the patient’s life. It is crucial that the FP has the scientific knowledge for the management of the disease and that they integrate it into the patient’s psychosocial context, advising him and supporting him in sharing his decisions.
Keywords : ARCAPA; Congenital heart defects; Patient-centered care; Primary health care; Case report.
