[Natural course of an aortic coarctation in a child]. / Évolution naturelle d'une coarctation de l'aorte de l'enfant.

Aortic coarctation is a frequent congenital heart disease that presents in form of two entities, the infant type and the child or adult type. The infant type is the most frequent and manifests acutely by heart failure or shock. The second one shows a slow and progressive course with no or few initial symptoms. For that reason, the diagnosis is usually missed until symptomatic arterial hypertension or hypertrophic cardiomyopathy develop. We report the case of an initially asymptomatic boy in whom the development of an aortic coarctation could be precisely documented over a period of 10 years by repeated bidimensional and Doppler echocardiography. The patient underwent successful balloon angioplasty of the aortic isthmus and stent implantation. This case points out the importance of strict follow-up in any young child in whom an even mild anomaly of the aortic arch is detected. This is critical in order not to miss an aortic coarctation that influences significantly morbidity in adulthood.

La coarctation de l'aorte est une cardiopathie fréquente qui se présente sous deux formes distinctes, celle du nourrisson et celle du grand enfant ou de l'adulte. La première, plus fréquente, se manifeste de façon aiguë par une défaillance cardiaque, voire un choc cardiogénique alors que la seconde est d'évolution lente et paucisymptomatique. Pour cette raison, elle reste habituellement méconnue jusqu'à l'apparition d'une hypertension artérielle ou d'une cardiomyopathie hypertrophique symptomatique. Nous rapportons le cas d'un garçon présentant initialement des structures aortiques de petit calibre chez qui l'évolution d'une coarctation de l'aorte a pu être documentée de façon précise sur une période de 10 ans. L'échocardiographie bidimensionnelle et Doppler répétée a permis de montrer le développement de la sténose de l'isthme aortique alors que le patient ne présente pas de signes d'appel cliniques. Il bénéficiera de l'angioplastie au ballon de l'isthme aortique et de la mise en place d'un stent. Ce cas met en avant l'importance d'un suivi rigoureux des patients chez qui une anomalie, même très légère, de l'arc aortique est mise en évidence dans la petite enfance de façon à ne pas méconnaître une coarctation de l'aorte qui conditionne la morbidité de l'adulte de façon significative.

Growing up with Idiopathic Pulmonary Arterial Hypertension: An Arduous Journey.

Idiopathic pulmonary arterial hypertension (IPAH) is an uncommon and severe disease. We report the case of a 7-year-old boy investigated for cardiac murmur and exercise intolerance. Pulmonary hypertension (PH) was suspected at clinical examination and confirmed by echocardiography and cardiac catheterization. This case of pulmonary hypertension was classified as idiopathic given the negative etiological investigation. Vasoreactive testing with oxygen and nitric oxide was negative. Therefore, treatment with sildenafil (1.4 mg/kg/d) and bosentan (3 mg/kg/d) was initiated. This allowed the stabilization of, but not a decrease in, pulmonary artery pressure for the next 5 years, during which the patient's quality of life was significantly reduced. At a later follow-up, the estimated pulmonary pressure was found to have increased and become supra-systemic, with a consequent deterioration in the child's condition. This led to the decision to enter him into a clinical trial that is still ongoing. Idiopathic pulmonary arterial hypertension is a severe disease that can present with non-specific symptoms, such as asthenia and exercise limitation, which are important not to trivialize. The disease is associated with significantly decreased quality of life in affected children and carries a high burden in terms of mortality and morbidity. The current knowledge about IPAH in children is reviewed, with a particular focus on the future prospects for its treatment and the related quality of life of patients.