sildenafil-citrate and Cyanosis

In recent years, there has been a rise in the number patients with CHD surviving into adulthood. Many have complications related to their CHD or its treatments, outside the heart, including ocular abnormalities. The objective of this review is to highlight the ocular abnormalities that occur in adults with CHD, either from their condition or related to the common drugs prescribed to manage it. In particular, we reviewed the effects of cyanosis, coarctation of the aorta, endocarditis, and the side effects of Sildenafil and Amiodarone. A change in the retinal vasculature is a common observation with cyanosis or coarctation of the aorta. Occlusion of the retinal vessels may also be observed in cyanotic patients, as well as those with infectious endocarditis. Sildenafil has established ocular side effects; here they are explored in the context of therapy for pulmonary hypertension. Similarly, Amiodarone has established ocular risks, which are summarised. The high prevalence of ocular consequences in adult CHD patients reinforces the need for knowledge of the risks involved and for frequent ophthalmological screening where appropriate.

Topics: Adult; Amiodarone; Aortic Coarctation; Cyanosis; Endocarditis; Eye Diseases; Heart Defects, Congenital; Humans; Retinal Vessels; Sildenafil Citrate

Methylmalonic acidemia or aciduria (MMA) is an inborn error of metabolism that results in the accumulation of methylmalonic acid in blood with an increased excretion in urine. MMA usually presents in early infancy and its effects vary from mild to life-threatening. The clinical symptoms mainly include vomiting, dehydration, hypotonia, developmental delay, and failure to thrive. An association between pulmonary arterial hypertension (PAH) and MMA has been rarely reported. In the present work, the authors report a 16-month boy, who was admitted to the Pediatric Department for cyanosis and fever. He had a family history of primary pulmonary hypertension in a sister. The echocardiography showed a mild pericardial effusion and PAH. The metabolic screening led to the diagnosis of MMA. The condition of the baby worsened rapidly- and he died a few days later. Physicians should be aware about this atypical presentation of the disease, which can be fatal if not diagnosed and managed promptly.

Topics: Amino Acid Metabolism, Inborn Errors; Cyanosis; Familial Primary Pulmonary Hypertension; Fatal Outcome; Fever; Humans; Infant; Male; Methylmalonic Acid; Renal Insufficiency; Sildenafil Citrate; Tachycardia; Vasodilator Agents

Topics: Cyanosis; Ductus Arteriosus, Patent; Female; Humans; Hypertension, Pulmonary; Osteoarthropathy, Secondary Hypertrophic; Sildenafil Citrate; Vasodilator Agents; Young Adult

Unilateral absent right pulmonary artery is a rare developmental anomaly that usually presents in late childhood and adolescence as recurrent respiratory tract infections, dyspnoea and haemoptysis. We report a case of a 2-day-old baby with respiratory distress and differential cyanosis. Echocardiogram showed pulmonary hypertension with absent right pulmonary artery. The findings were confirmed by CT angiogram. The baby improved with pulmonary vasodilators and antifailure medications.

Topics: Computed Tomography Angiography; Cyanosis; Diagnosis, Differential; Echocardiography; Humans; Hypertension, Pulmonary; Infant, Newborn; Male; Pulmonary Artery; Respiratory System Abnormalities; Sildenafil Citrate; Vasodilator Agents