Case Report
A 26-year-old female patient was referred to our tertiary center because her dyspnea did not decrease 2 months after percutaneous balloon mitral valvuloplasty (PBMV) for rheumatic mitral stenosis. She had no family history of CHD. At the admission, her functional capacity was NYHA-III. Arterial blood pressure was 125/80 mmHg, peripheral oxygen saturation was 97%, and heart rate was 75/beats per minute. There was a 2/6-degree systolic murmur on auscultation besides a wide split-second heart sound (S2). ECG showed sinus rhythm and did not show any specific changes. Two months ago, just after the PBMV procedure, mitral valve area (MVA) was reported as 1.8 cm2. The patient was admitted to the emergency department several times with palpitation attacks after discharge and had some transthoracic echocardiography (TTE) reports performed by different cardiologists during this period. Her MVA measurements ranged between 1.2 cm2 and 1.9 cm2 on TTE.
On our TTE examination, ejection fraction (EF) was normal, mitral valve area (MVA) was 1.72 cm2, and we observed a secundum type atrial septal defect (ASD) accompanied by dilatation in the right heart chambers (Figure-1) with moderate tricuspid regurgitation (TR). Pulmonary arterial systolic pressure (PASP) was also measured as 48 mmHg. Transesophageal echocardiography (TOE) showed that MVA was 1.65 cm2 by pressure half time on 2-D evaluation and a diastolic D-sign on the interventricular septum (IVS) (Figure-2). With the 3-dimensional Multiplanar reconstruction (MPR) measurement of MVA was 1.63 cm2 (Figure-3). The mitral valve gradient was 9/5 mmHg, and as such, it was evaluated as mild mitral stenosis. Moderate TR was observed, PASP was 50 mmHg, and tricuspid annular diameter was 37.5 mm. The interatrial septum was aneurysmatic and a 1.22x0.67 cm of atrial septal defect was observed with the 3-D examination (Figure-4), and left to right shunt was seen through this defect. Also, the patent foramen ovale (PFO) tunnel was observed. Although the drainage of the left upper, left lower, and right lower pulmonary veins into the left atrium were observed, a cardiac MRI evaluation was planned because the right upper pulmonary vein drainage could not be adequately evaluated. Interestingly, the patient applied PBMV two months ago, and she was assessed with TOE before; no ASD finding was found in that period. In this case, we concluded that the current ASD is an iatrogenic defect secondary to the PBMV.
On cardiac MRI, dilatation in the right chambers, diastolic D-sign on IVS, secundum type ASD (Qp/Qs ratio: 2.82) were also observed, consistent with TOE. We found that the right upper and middle lobe pulmonary veins drained into the superior vena cava on cardiac computed tomography (CT), and it was interpreted as a partial pulmonary venous return anomaly (Figure-5). Then, we performed a cardiac catheterization, and our main findings are followings; 1) Qp/Qs: 6.28, 2) pulmonary vascular resistance was 1.04 wood unit, 3) systemic vascular resistance was 10,42 wood unit, 4) pulmonary capillary wedge pressure was 7 mm Hg, 5) mean pulmonary artery pressure was 15 mm Hg. Hence, the results of catheterization consisted of left-to-right shunt. At this stage, the evaluation made with the patient gained importance, and the heart team decision was determined because the patient was young and considering pregnancy.
Surgical treatment for pulmonary venous return anomaly and ASD was decided in the heart team council for the patient. A follow-up with medical treatment was planned for the mitral valve depends on low pulmonary capillary wedge pressure. The PAPVR was repaired by robotic intracardiac routing using the da-Vinci system, and the ASD was closed successfully.
In the TTE evaluation performed 2 months after the surgery, the right heart chambers were in normal size (Figure-6), MVA was 1.63 cm2, mitral valve gradient was 9/6 mmHg, mild tricuspid regurgitation, and PASP was 24 mmHg. The patient was symptom-free, and her functional capacity was NYHA-I.