Abstract
In this case, we reported an old female with a rare combination of the
Scimitar Syndrome and pulmonary artery aneurysm, and she presented with
chest pain, fatigue and shortness when admitted in our hospital. Further
cardiac examination including echocardiography and computed tomography
angiogram were performed and revealed these rare anomalies. Surgical
procedure was carried out to cure the patient. This patient stayed in
ICU for few days without any major complications and discharged from
hospital after 10 days.
Keywords : Congennital heart disease
A 62-year-old female was referred to department of cardiovascular
surgery in our hospital presented with spontaneous chest pain, fatigue
and shortness of breath for 2 months. She had been found of
dextroposition of the heart and heart murmur for over 30 years, since
remained asymptomatic all along except catching cold easily, treatment
was not carried out before. Physical examination revealed a little
displacement of apex beat site to the right, and grade III systolic
murmur located at the left margin of the sternum, second intercostal
space.
Among further cardiac examination, transthoracic echocardiography (TTE)
revealed severe dilation of pulmonary artery accompanied with pulmonary
valve stenosis, and the inferior venous cava (IVC) was significantly
widened. Computed tomographic images demonstrated hypoplasia of right
pulmonary artery and lung. Computed
tomography angiogram (CTA) indicated significant dilatation of pulmonary
artery trunk and the left pulmonary artery with a maximum diameter of
6.0 cm (Figure 1, panel A), three-dimensional reconstruction visually
demonstrated the aneurysmal dilatation of pulmonary artery (Figure 1,
panel B). Two branches of left pulmonary vein merged into the left
atrium, while right pulmonary veins were not found to enter into the
left atrium (Figure 1, panel C). The CTA image demonstrated the right
common pulmonary vein directly merged into the inferior vena cava above
the diaphragm, while the diameter of the IVC was significantly enlarged
(Figure 1, panel D).
Open heart surgery through middle sternotomy under cardiopulmonary
bypass was performed for the correction of scimitar vein. Autologous
pericardial patch was used to create an intra-atrium tunnel to baffle
the scimitar vein into left atrium via the artificial atrial septal
defect. In addition, the enlarged pulmonary artery was repaired close to
the normal diameter. Intraoperative transesophageal echocardiography
revealed the flow of inferior vena cava and scimitar vein were constant
and scimitar vein merged into the left atrium smoothly through the
atrial septal defect, which proved operation was successful. This
patient stayed in ICU for few days without any major complications and
discharged uneventfully after 10 days.
The scimitar syndrome is a congenital cardiovascular defect consisted
following anomalies: partial or total anomalous right pulmonary venous
drainage to the inferior vena vein with variable degrees of right
pulmonary artery and lung hypoplasia; one or more anomalous abdominal
aortopulmonary collateral to the right lung, usually right lower lobe;
and variable degrees of dextroposition of the
heart1~3. The syndrome is usually
appeared with other defects, atrial septal defect (80%) is the most
common comorbidity, following is patent ductus arteriosus (75%),
ventricular septal defect (30%) and pulmonary vein stenosis
(20%)4. We reported this rare case of the scimitar
syndrome combined with aneurysmal dilatation of the pulmonary artery.
Further speculation, long-term overload of right heart volume, existence
of aortopulmonary collateral and mild pulmonary valve stenosis common
caused the significant dilation of the pulmonary artery, and finally
lead to symptoms of heart failure.