2 CASE REPORT
A 36-year-old woman presented to the local hospital due to left
abdominal pain. The pain was persistent and can’t be relieved. She had
vomited twice without fever and regurgitation of sourness. The X-Ray
revealed that there was incomplete intestinal obstruction. There was not
significant relief after the tratements of herb retention enema,
anti-infection and fluid infusion, so she presented to our hospital for
further tratements. She had a temperature of 38.1 degrees celsius
without shivering.
She knew that she had a congenital heart disease without the definite
diagnosis when she was ten years old. she had occasional chest pain
after 30-years old. Two months ago, the right extremities were numb,
powerless, and the right eye was amaurotic. She had been constipated for
two months. She had a cesarean section in 2009.
Transthoracic echocardiography revealed that there is
a large cyst with many septums,
which connected with the left atrium. The connection diameter is 2.2
centimetres. Color Doppler showed the flow converged between left atrium
and the cysts. Due to the cysts’ irregular shape and large volume, it
couldn’t be detected in a view (Figure 1).
There
was also PLSVC connecting to the left atrium(LA) . Retrograde flow from
the LA to PLSVC acrossing the bridging innominate vein to the right
superior vena cava(RSVC)
was
found. Suprasternal notch view revealed that the PLSVC
with
retrograde flow drained into
bridging
innominate vein (Figure 2). In
agitated saline contrast
echocardiography, the right atrium (RA) and right ventricle(RV) was
filled with air bubbles, but not the cysts and left atrium. This finding
demonstrated that the PLSVC didn’t connect to right atrium directly or
through the CS. And In contrast-enhanced echocardiography of the left
heart (including left ventricular opacification [LVO]),an
ultrasound contrast agent filled the cysts and LA with some area
filling-defect was observed,some was slow,in a swirl-like shape
(Figure 1). Slow hemodynamics is a risk factor for thrombosis.
The multi-slice spiral CT showed that there was a huge dilated CS. The
dilated CS was separated in many
cysts(composed
of the great and small cardiac veins). The CS was ended in a long blind
pouch with atresia of its ostium. There was an UCS about 2.4cm as an
outlet for the dilated CS. PLSVC connecting to LA and draining into the
bridging innominate vein was
observed (Figure 3). The three-dimensional reconstruction CT shows the
whole morphology in cardiac anomaly (Figure 4).
According to all above, we considered that she had CSOA, the dilated CS
drained into the LA through UCS, which was as a window to the left
atrium for all cardiac vein to return. A PLSVC
connecting
to left atrium with retrograde flow drained into bridging innominate
vein.
3 DISCUSSION
The CS is located posteriorly in the left atrioventricular groove. It
opens into the RA through the orifice, which is located in the posterior
and inferior wall of the RA and superior to the tricuspid valve. The
dilation of CS is an important sign of many cardiac anomalies, such as
PLSVC, UCS, Partial anomalous pulmonary venous
return, coronary arterio-venous
fistula, elevated right atrial pressure. CSOA is a rarely anomaly with a
prevalence of about 0.1%. There are three types of cardiac venous
drainage in patients with CSOA. Type (A): A PLSVC connected to the CS.
Type (B): Venous drainage through the UCS to the LA. Type (C): Anomalous
CS drainage (asterisk) into the RA through an accessory vein, such as a
small cardiac vein or a Thebesian vein.2 This case
belongs to the type B. A PLSVC results from persistent patency of the
left anterior cardinal vein draining into a dilated coronary sinus.
PLSVC is the most common congenital thoracic venous anomaly with a
prevalence of 0.3–0.5% in general population.3Almost 40% of patients with PLSVC can have a variety of associated
cardiac anomalies. CSOA with a PLSVC drained into left atrium is a rare
cardiac anomaly.4 In this case, the patient had a
extremely dilated coronary sinus with an irregular shape, the LA was the
only alternative pathway of the dilated CS,5,6 and the
PLSVC connected to the LA with retrograde flow, which was ignored
probably in transthoracic echocardiography. The dilated CS was so large
with irregular shape, there was the
probability of some tiny thrombus in it causing embolism in any
vessel.
This patient has a renal infarction, the source of the embolus is most
likely from the dilated CS and
LA.
In
the presence of a
left
persistent vena cava with a relatively normal diameter innominate vein,
CSOA via retrograde drainage should be suspected. Multimodality imaging
such as,
transthoracic
echocardiography, transesophageal echocardiography, contrast
echocardiography, three-dimensional echocardiography, CT, or magnetic
resonance imaging is needed to diagnose this anomaly. It is imperative
to identify this anomaly preoperatively because ligation
of
PLSVC can lead to interruption of coronary venous flow and,
subsequently, myocardial congestion and ischemia.7-10The relationship between CSOA plus LSVC and other cardiac anomalies is
unclear. Other cardiac malformations coexist in approximately 56% of
reported cases.11 This patient had no other cardiac
anomalies, so she only accept anticoagulant therapy. She has now been
discharged from the hospital and receives regular follow- up care.