De
Buckes syndrome – TOF with unilateral absence of a pulmonary artery (UAPA) is a
rare condition with an estimated prevalence of 1 in 200,000 young adults .
Most commonly, UAPA occurs in conjunction with cardiovascular abnormalities
such as tetralogy of Fallot (TOF) coarctation of the aorta, VSD, subvalvular
aortic stenosis, transposition of the great arteries (plus VSD or pulmonary
stenosis), Taussig-Bing malformation and coarctation, congenitally corrected
transposition and pulmonary stenosis, scimitar syndrome. Patients with isolated
UAPA can remain asymptomatic into late adulthood but usually report symptoms
such as dyspnea or chest pain or suffer from hemoptysis or recurrent
infections. Diagnosis can be difficult due to the rarity of the condition and
its nonspecific presentation. We present a case of a 5month old child who
presented with TOF with Right pulmonary artery stenosis and absent left
pulmonary artery, with typical findings on chest radiograph, angiographic
features and treatment discussed.
Case
Report: A
five months old male child was referred to Sri Jayadeva Institute of
Cardiovascular Science and research for cardiac evaluation. He was born to
non-consanginous parents with normal pregnancy and normal delivery. He
presented with history of incessant feeding and cyanosis while feeding and
crying. On physical examination, the patient had cyanosis with SpO2 of 67%.
There was no respiratory distress at rest. His weight was 3 kgs (just below the
5th percentile for his age). Heart rate was 104 beats/minute and blood pressure
was 100/55 mmHg. Cardiovascular examination revealed normal peripheral pulses,
the second heart sound was single and Grade 3/6 Ejection systolic murmer. The
electrocardiogram showed sinus rhythm, right axis deviation and right
ventricular (RV) hypertrophy. Chest X-ray showed mild cardiomegaly, with normal
bronchovascular markings on right side and absent bronchovascular markings on
left side. Read more..........
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