Comment on sickle cell disease and left ventricular hypertrophy☆

Sickle cell disease (SCD) is a monogenic disease that causes a significant reduction in life expectancy due to the presence of chronic anemia, acute painful episodes and organic injuries. The most common chronic injuries are cardiopulmonary complications, such as pulmonary hypertension (HP), chronic lung disease, diastolic dysfunction and congestive heart failure (CHF), which are manifested mostly in adulthood. These complications are associated with early mortality. With the improvement of the healthcare system and greater access to proper treatment, there is a growing interest in identifying complications as early as possible by evaluating patients in childhood and adolescence in order to improve morbidity and mortality rates.1 Patients tend to have increased left ventricular (LV) stiffness with consequent left ventricular hypertrophy (LVH) and progression to increased high pulmonary blood pressure. Another complication due to LVH is diastolic dysfunction, an early marker of cardiac impairment preceding CHF that is frequently found in adults with SCD.2 The pathophysiologic mechanism of HP in individuals with SCD has not been well defined but is probably multifactorial. Potential etiologic factors include hemolysis that interferes with nitric oxide-mediated vasodilatation, LV dysfunction, pulmonary thromboembolism, airway hyperreactivity, and sleep-disordered breathing.3 A high cardiac output and high pulmonary blood flow, combined with intravascular hemolysis promote these structural changes.4 The incidence of HP in the pediatric population ranges from 11% to 30%. In a paper published by Colombatti et al., there is confirmation that HP starts early in children showing the