دکتر حسن متقی مقدم

Inadequate pulmonary blood flow in the tetralogy of Fallot (ToF) can lead to the development of major aortopulmonary collaterals, interfering with surgical repair.

We aimed to depict the crucial role of cardiac catheterization before the surgical intervention of pediatrics undergoing ToF total correction.

This retrospective cohort study was conducted on consecutive pediatric patients under two years of age who underwent ToF total correction surgery. All of the patients were diagnosed with ToF with doubled times trans-thoracic echocardiography, and the patients with high suspicion of the presence of major aortopulmonary collateral arteries (MAPCAs) underwent cardiac catheterization before ToF total correction surgery. Conversely, if the catheterization approach failed, they underwent ToF total correction surgery simultaneously with MAPCA closure. On the other hand, the patients with no evidence of MAPCA in the echocardiographic findings (as considered undiagnosed patients) underwent ToF total correction surgery.

Two hundred fifty eligible pediatrics who underwent ToF total correction surgery participated. The ICU stay (P < 0.001), hospital stay (P < 0.001), and weaning time from mechanical ventilation (P < 0.001) in patients with correct diagnosis before the ToF surgery were statistically lower than misdiagnosed open MAPCA patients who underwent the surgical approach of the ToF group. Some intra-operative and postoperative parameters showed no statistical differences between patients in the two groups. We had three mortality in the patients whose trans-thoracic echocardiography (TTE) findings were undiagnosed as there was no evidence to rely on the presence of open MAPCA.

Based on this study's findings, we strongly recommend that all ToF patients undergo cardiopulmonary catheterization before the surgical approach.

Unilateral absence of a pulmonary artery (UAPA) is an uncommon anomaly with an estimated prevalence of 1 in 200,000 young adults and a diverse clinical presentation. It may be diagnosed as an isolated anomaly or in combination with other congenital heart defects. The purpose of the present study was to investigate the clinical course and characteristics of forty patients with UAPA in whom the diagnosis was first established in childhood or adulthood.

From January 2002 through April 2021, 40 patients with unilateral absence of one pulmonary artery were diagnosed at our institution. We retrospectively reviewed the medical records of these patients in detail and elicited information covered patients' demographics, associated anomalies, imaging studies and operation data.

All forty patients were between 2 months and 25 years old (mean age, 6.45 years) at first presentation, weighing 4.70-59.00 kg (mean weight, 19.17 kg) and twenty five patients were female (62.5%). Absent left pulmonary artery (LPA) was found in 60% of cases (24 patients) and 40% had occult right pulmonary artery (RPA). Right-sided aortic arch was found in 4 patients (10%). Tetralogy of Fallot was diagnosed in 16 patients while complex cardiac anomalies were found in 13 cases.

clinicians should consider the undiagnosed cases of UAPA especially in adults with suspicious symptoms such as unexplained hemoptysis. Applying different available imaging techniques for early diagnosis and treatment of isolated UAPA cases are important to prevent the devastating complications of pulmonary hypertension and massive hemoptysis in long term.