Discussion
To the best of our knowledge, this is the first case in which partial
anomalous pulmonary venous return (PAPVR) anomaly, iatrogenic ASD and
rheumatic mitral stenosis were found together. Anomalous pulmonary
venous return (APVR) is a rare congenital anomaly seen between 0.4% and
0.7% in the autopsy series (3). The main pathophysiologic problem in
APVR is the direct or indirect opening of the pulmonary veins into the
right atrium (1,4). While one or more pulmonary veins are defective in
the partial type, all of the pulmonary veins are unrelated to the left
atrium in total anomaly (5). PAPVR is most commonly encountered in the
supracardiac form in which the right upper pulmonary vein drains into
the right atrium or superior vena cava (6), as in our case. The abnormal
pulmonary venous return may be supracardiac, cardiac, infracardiac, or
mixed type. Although an anomaly in the drainage of a single vein usually
does not cause hemodynamic problems, an anomaly in more than one vein or
other congenital or acquired diseases may cause the symptoms to occur
(4,7). PAPVR is often accompanied by atrial septal defect, which has
been reported to be 82% in some publications (8). There are also some
publications on its association with rheumatic mitral stenosis (9,10).
PAPVR usually has a silent clinical course and varies depending on the
degree of left-to-right shunt and other associated cardiac anomalies
(11,12). The clinical problem in our patient was accompanying mitral
stenosis. Diagnosis of PAPVR was missed during the evaluation period for
mitral stenosis, and therefore the treatment was determined as
percutaneous mitral balloon valvuloplasty. However, with the effect of
iatrogenic ASD due to PBMV, unfortunately, the volume load in the right
chambers increased. Thus, the patient’s symptoms continued as before
PBMV, although mitral stenosis was treated.
The incidence of ASD following PBMV ranges from 4% to 53% (13). This
high variability is related mainly to the diagnostic method used. In a
study by Arora et al., in which TOE was used in the assessment, the rate
of ASD was observed as 92% immediately after PBMV, 80% after 72 hours,
and 10% in the evaluation 3 months later. So, most of the iatrogenic
ASD close spontaneously (14).
In our case, the iatrogenic atrial septal defect was too large to be
associated with only transseptal puncture. Most likely, the
inappropriate withdrawn of the percutaneous mitral balloon delivery
system damaged aneurysmatic septum and caused it to tear. Otherwise,
such a large atrial septal defect was unlikely to be overlooked in TOE
assessment before PBMV. The abnormally connected pulmonary vein was
drained to the superior region of SVC, so it cannot be clearly detected
with TOE. Also, the presence of diastolic D-sign has forced us to find
the cause of a volume load other than iatrogenic ASD. As a matter of
fact, while the diastolic d-sign indicates a significant volume load, it
was not possible to explain this with only 2-monthly iatrogenic ASD.
however, we would expect to see systolic D-sign secondary to pressure
increase in patients with severe mitral stenosis diagnosed late.
The crucial question to be answered in our case was which strategy
should be chosen in a patient with diastolic-D sign, dilatation in the
right heart cavities, mild mitral stenosis, and especially planning
pregnancy. Cardiac MRI and CT demonstrated a partial pulmonary venous
return anomaly associated with an isolated single vein located
superiorly. In cardiac catheterization, significant left-right shunt was
observed, and pulmonary capillary wedge pressure was within normal
limits. These findings proved that mitral stenosis was not serious and
the main problem was left-to-right shunt. It was decided by the heart
team to perform PAPVR and ASD repair with robotic method. Close
follow-up was planned for mitral valve stenosis. Thus, the patient would
have the chance to be treat with PMBV or mitral valve replacement after
pregnancy. In addition, a treatment without sternotomy would have
reduced the risk of cardiac surgery that could be performed in the
follow-up. After all, the patient was successfully treated with the
robotic surgery (da-Vinci system), and normalization was observed in the
right heart chambers and pulmonary artery pressure in the second month
control TTE.
The take-home messages that we want to give in our patient, who was a
very important example of the individualized treatment option:
1- It should never be forgotten in clinical practice that an acquired
disease such as rheumatic mitral stenosis may be accompanied by a
congenital disease.
2- Even if the partial pulmonary venous return anomaly is isolated, it
should always be kept in mind in the causes of volume overload and
pulmonary hypertension in adults.
3- Iatrogenic atrial septal defect sizes can sometimes be much higher
than expected. The operation team should definitely check the IAS just
after the procedure.
4- Some individual indications such as pregnancy request may force the
physician to completely review patient management, surgical indications
and methods.
5- The optimum treatment option for each patient should be evaluated in
their own circumstances.