2.1. Presentation of Case 1
The first case was a 49-year-old female. The patient was diagnosed with pulmonary problems around 14 years ago. She also had endoscopy examination two years ago.
She underwent the workup for diagnosis of the cause of the pulmonary problem. The patient had dyspnea on exertion (DOE) with class II functional symptoms according to New York Heart Association (NYHA) functional classification. Other complications included hypertension and diabetes mellitus. The patient was taking the following medicines: Lantus, Zipmet (sitagliptin/metformin), atorvastatin, metoral, losartan, gliclazide, aspirin, peptazol, domperidone and vitamin B1. A diastolic S1-S2 murmur was heard on auscultation.
Transesophageal echocardiography demonstrated a normal sized left ventricle without hypertrophy and a mildly reduced systolic function. Left atrium was normal in echocardiography and right atrium was enlarged. Right ventricle was moderately enlarged and demonstrated a slightly reduced systolic function. Mitral valve appeared normal and without stenosis, however a mild mitral regurgitation was observed. Aortic valve was normal and without stenosis or aortic insufficiency. Mean pulmonary arterial pressure was 30 mmHg. Multislice computed tomography (MSCT) showed scimitar vein. Surgical repair was recommended to correct the left-to-right shunt, as a result of pulmonary venous drainage to the inferior vena cava.
Reparation of the Scimitar vein was performed according to the regular method for correction of Scimitar syndrome and anomalous vein was diverted from IVC to the left atrium through ASD. The patient was examined using echocardiography after operation. Correct routing of the anomalous vein to the left atrium without stenosis was observed.
2.2. Presentation of Case 2
The second case was a 16-year-old female. She had complaints about DOE. Patient underwent the workup for the diagnosis of the cause of the pulmonary problem. DOE was of functional class II according to the NYHA functional classification. Her blood pressure was 110/80 and pulse rate was 85/minutes. She was presented with systolic murmur on auscultation. The patient had no other apparent presentation of the condition.
The patient underwent angiography with the following significant observations: O2 saturation percent in the aorta, pulmonary artery (PA) and the superior vena cava (SVC) were 96, 85, and 78 mmHg, respectively. This amount was 87% for inferior vena cava (IVC) which suggests a left to right shunt from pulmonary vein to IVC (Figure 1). Other significant angiographic observations included Scimitar vein of right heart catheter and secundum type atrial septal defect (ASD) as observed by the left side catheter. The LV size and diastolic function was normal. However, a mild mitral regurgitation was observed. Mean pulmonary arterial pressure of this patient was 40 mmHg.
Figure 1.
Scimitar vein draining into inferior vena cava (IVC)
Transesophageal echocardiography demonstrated a normal sized left ventricle without hypertrophy. Left atrium was normal in echocardiography and right atrium was enlarged. Right ventricle was severely enlarged and with mild systolic dysfunction. Mild enlargement of the right atrium was observed. Mitral valve appeared normal and without stenosis with mild regurgitation. Aortic valve was normal and without stenosis or aortic insufficiency. Large secundum type ASD (1.7 cm) with a significant left to right shunt (QP/QS: 2.5) was observed. Right pulmonary vein was entered near the IVC entrance to the RA. Observations was consistent with the diagnosis of a large ASD and a Scimitar vein.
Multislice computed tomography showed scimitar vein and levocardia. Right (RAE) and left atrial enlargement (LAE), and central pulmonary artery dilatation were observed. Scimitar syndrome was noted as almost complete drainage of the right lung venous flow to the supra hepatic IVC. Only a small right sided subsegmental venous branch connects directly to the LA. All left pulmonary vein is drained into the LA.
The recommended treatment was surgical repair to correct the left-to-right shunt, as a result of pulmonary venous drainage to the inferior vena cava, and ASD closure. For this patient, reparation was also performed according to the routine approach for correction of Scimitar syndrome and anomalous vein was diverted from IVC to the left atrium through ASD. Echocardiographic examination of the patient after operation, demonstrated correct routing of the anomalous vein to the left atrium without stenosis.
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