To the editor: Scimitar syndrome is a rare congenital anomaly consisting in part of right pulmonary venous return to the inferior vena cava (IVC). The infantile variant is marked by a higher incidence of associated defects, heart failure, pulmonary hypertension (PH), and significant mortality. The patient with the pediatric/adult form is less severely affected. We report a rare case of an 18-year-old girl with atrial septal defect (ASD) and severe PH.
In August 2012, an 18-year-old girl was admitted because of upper abdominal pain. Previously, she had been well with subjectively normal exercise tolerance and no history of recurrent respiratory symptoms, shortness of breath, cyanosis,or palpitation. Cardiac systolic murmur in pulmonary area and cardiac enlargement were observed, and breath sounds were normal, with no crackles. A chest CT scan with multiplan reformation and three-dimensional reconstruction (Figure 1A-C) was performed and showed congenital combined heart and vascular anomalies. Cardiomegaly mainly in the right atrium and ventricle was observed. ASD (2 cm in width) was found. Main pulmonary trunk (2.9 cm) and left pulmonary trunk were obviously widened. Anomalous venous return was observed with a large sinuous scissural vein originated from the right pulmonary vein directed into the segmenta hepatis of the IVC. The IVC enlarged notably (5 cm in the widest point). The superior vena cava, left pulmonary vein and lungs CT scan examination were normal. A transthoracic echocardiogram (Figure 1D) demonstrated a large atrial ASD (3.3 cm), a dilated and hypertrophied right heart with severe tricuspid reflux (Vmax=3.1 m/s, PG=38 mmHg (1 mmHg=0.133 kPa)), abnormal drainage of right pulmonary venous flow into the IVC. The pulmonary artery systolic pressure was 43 mmHg.
Scimitar syndrome was first described in autopsy specimens in 1836.1 Partial or complete anomalous connection of the right pulmonary veins to the IVC is one of the main features of the syndrome. This patient presented upper abdominal pain related to anomalous venous return. The systolic murmur related to severe tricuspid reflux. Pulmonary hypertension may be attributed to left-to-right shunting from the ASD. Scimitar syndrome combined with ASD and PH is rare in adults.2 Only a few cases involving adults have been reported, and diagnosis remains uncertain until operation. Multiplan reformation and three-dimensional CT scan offer an accurate noninvasive diagnosis, avoiding cardiac catheterization and leading to a satisfying classification of this variant of scimitar syndrome.
1. Cooper G. Case of malformation of the thoracic viscera consisting of imperfect development of right lung and transposition of the heart. London Med Gaz 1836; 18: 600.
2. Shin DH. Atrial septal defect with total anomalous pulmonary venous return in an adult. Circulation 2011; 123: e612-e613.