Idiopathic arterial calcification of infancy is a rare autosomal recessive disease, characterized by deposition of calcium along the internal elastic membrane of arteries, accompanied by fibrous thickening of the intima which causes luminal narrowing. Here we are reporting a case of idiopathic arterial calcification of infancy in a Saudi female newborn of non-consanguineous pregnant woman who had polyhydramnios. The newborn baby had severe respiratory distress, systemic hypertension and persistent pulmonary hypertension of newborn. She was admitted to Neonatal Intensive Care Unit, where she was ventilated and proper treatment was provided. Molecular genetic testing was positive for mutations of ectonucleotide pyrophosphatase/phosphodiesterase1 gene which is reported in 80% of cases of Idiopathic arterial calcification of infancy. The baby died at about 5 month of age because of myocardial ischemia and cardiorespiratory arrest. Idiopathic Arterial Calcification of Infancy should be considered in any newborn who presented with persistent pulmonary hypertension of newborn, severe systemic hypertension and echogenic vessels on any radiological study. Calcifications of large and medium-sized arteries are important diagnostic finding.

Heart failure and preserved ejection fraction (HFpEF) is defined as heart failure symptoms and signs with a normal or near-normal ejection fraction (EF) with evidence of diastolic dysfunction. The few Middle Eastern studies that have been conducted were designed to compare patients with heart failure reduced ejection fraction (HFrEF) and HFpEF.The aim of this study was to study Saudi patients with HFpEF who presented with acute heart failure, and define their clinical characteristics and the signs and symptoms of heart failure, echocardiographic findings and medications at admission and at hospital discharge.MethodsThis is a prospective observational study in which patients were included following an acute heart failure presentation with N-terminal pro-BNP (NT-proBNP) > 300 ng/L and left ventricular ejection fraction (LVEF) > 50%. They were admitted to the coronary care unit of king Saud medical city from the period of March 2015 to September 2015.Results114 patients were enrolled in the study and assessed at acute admission. Of these, 4% died on day one of admission.The mean ± SD age of 109 included patients was 59 ± 8 years and 55% were women. Hypertension (64%), dyslipidemia (76%), atrial tachyarrhythmia (38%), prior heart failure (33%) and anemia (35%), median NT-proBNP was 2490 ± 125 ng/l at admission. Mean (LVEF) was 61 ± 3, mean LV mass index was 118 ± 11, mean E/e′ was 12.2 ± 2, and left atrial volume index was 47 ± 7 mL/m2. Mean global left ventricular strain was −13.5 ± 1.5. At discharge the majority of patients were still symptomatic with high NT-proBNP 542 ± 266.ConclusionsPatients with HFpEF were old with slight female dominance, a high rate of hypertension, diabetes, dyslipidemia and much comorbidity. LVEF was preserved despite depressed left ventricular longitudinal and diastolic functions with high filling pressure. At discharge the patients were still symptomatic calling for further research to reach the best strategy for proper management.

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