Introduction: Primary pulmonary arterial hypertension (PPAH) is a rare disease, at most times underdia-gnosed because of its unspecific clinical features, which in medical practice raise serious problems of establishing a diagnosis, thus the evolution remains unpre-dictable and the disease prognosis is reserved. In pediatric cardiology, PPAH is most frequently caused by congenital heart defects, which have not been solved in a timely manner.

Methods: We would like to present the case of a 13 years and 3 months old adolescent boy, referred to our practice on his parents’ request for fatigue (present since early childhood) and ECG abnormalities which have been found on a routine pediatric examination. The patient has stage I obesity.

Results: On the first pediatric cardiology assessment in our practice, we have highlighted the typical aspect of a right ventricle pressure overload with ischemic changes in the V1-V4 derivation on the surface elec-trocardiogram. The echocardiography showed a re-acted right ventricle, with an anfractuous structure, a flattened interventricular septum and medium tricus-pid insufficiency. Advanced imagistic investigations (MR-Angiography) argue against an arrhythmogenic right ventricular dysplasia, and cardiac catheterization showed hemodynamic alterations which are suggestive for PPAH without any reactivity to the administration of a pulmonary vasodilator; this indicates the need to initiate treatment with endothelin receptor antagonists, with a slight improvement of the clinical status and effort tolerance.

Conclusions: We would like to bring this case to your attention, in order to urge you, to refer any patient with a high level of fatigue, which is not in accord with his clinical status, to a pediatric cardiology practice, be-cause PPAH is a pathology with a difficult manage-ment plan, because of the lack of clinical study based evidence, the low incidence of this pathology in chil-dren without congenital heart defects, the difficulty of establishing the diagnosis, the etiology and supportive treatment.