Pulmonary Hypertension and the Implications for Care of the Aerodigestive Patient

Several of the comorbid conditions associated with aerodigestive disorders (AD) perpetuate lung injury and declining functional status, and thereby pose a significant health risk to children. One complication in particular pulmonary hypertension (PH) can occur as a primary condition in isolation or can arise as a condition secondary to altered vascular and lung development. PH is a multifactorial disease with complex pathophysiological mechanisms, with airway inflammation, hypoxic vascular remodeling, and rarefaction all considered contributing factors. Despite the distinct pathogenetic characteristics of these entities, they can all lead to severe PH, resulting in an increase in right ventricular afterload and, as a common final outcome, right-sided heart failure and death. Regardless of the underlying pathophysiological causes, PH diagnosis has a significant burden in terms of mortality and morbidity, as high as 40%. In its classification of PH in 2011, the Panama Group highlighted developmental disorders of the lungs as a significant cause of childhood PH. The developmental lung disorders with highest risk of PH include bronchopulmonary dysplasia (BPD), congenital diaphragmatic hernia (CDH), trisomy 21, which are common comorbidities in aerodigestive patients. There is increasing evidence that the dysmorphic pulmonary vascular beds in developmental lung disorders such as these combined with ongoing pulmonary insults can lead to the development of PH. As our knowledge and treatment of AD, PH, and associated comorbidities improve, children are living longer than ever before, necessitating the need for repeated sedation and anesthesia. Children with PH are at disproportionately high risk of perioperative complications, and tailored interventions are vital to improving patient outcomes.