We present a 45 years old woman with known congenital heart defect, who was transferred to our hospital with right atrial tumour suspicion and an unrecognized BCS.
A surgical closure of an atrial septal defect (ASD) was performed when she was 8 years old. After childbirth in 2008, she was treated with heparin for pulmonary embolism, followed by a short period of warfarin therapy (a few months). An echocardiographic examination (ECHO) was performed in 2009, after the patient complained of a certain effort intolerance, and a discrete leg edema. A recanalization of ASD was excluded, and the finding was described as within normal limits.
During the preceding 6 months, she had some serious clinical symptoms: a more severe dyspnea, cough, effort intolerance, ascites and leg swelling. She was admitted to a county hospital. The differential diagnosis was focused on heart failure, lung disease, and occult neoplasm. ECHO revealed a tumorous process in the right atrium 2.7 × 1.7 cm attached to the atrial septum (Fig. 1). She was transferred to our institution for further investigation and treatment.
At admission, the patient’s clinical examination revealed a markedly distended abdomen with prominent superficial veins, bilateral pedal edema and hepato-splenomegaly. There was no jaundice. Clinical presentation was highly suspicious for BCS. ECHO (transthoracic and trans-esophageal), followed by MRI, revealed a membrane, dividing IVC inflow and right atrium, without communication (Fig. 2).
Percutaneous intervention was indicated. The patient was primed with 300 mg of Clopidogrel and 325 mg of Aspirin. Venous access was obtained via 6 French sheaths in the right femoral vein. Unfractionated heparin (5.000 units) was given. Venography was performed via pigtail catheter. It revealed a short, completely occluded segment (thick web) between the right atrium (RA) and IVC. After an unsuccessful attempt to puncture the membrane with a distal tip of an Amplatz stiff wire (J curve, 0,035 Cook Medical, Bloomington, IN, USA), the thick web was punctured with a curved needle through a transseptal introducer sheath (Mullins Sheath 8 Fr and Brokenborough needle 18 gauge, Medtronic, Minneapolis, MN, USA), in the same manner as we would puncture the interatrial septum. After confirming the position of the needle by contrast injection, the sheath was advanced over the needle into the RA, beyond the occlusion. Then a 0.032 J-tip wire was negotiated across the web and was parked in the right subclavian vein. The membrane was first dilated with an available 6,0/20 mm non compliant coronary balloon (NC Emerge, Boston Scientific, Boston MA, USA), with 16 atm, followed by dilatation with a bigger non-complined peripheral balloon16/20 mm with 20 atm (Atlas Gold, Bard Vascular, Tempe AZ USA), with poor result. Sequentially, several balloon dilatations with 30/40 mm semi-compliant balloon (Z-med II, Numed-Braun, Bethlehem, PA, USA), with 4 atm pressure was performed, resulting in immediate recoil. Satisfactory result was achieved after the inflated big balloon was sharply withdrawn through the membrane (a “Rashkind-like” manoeuvre). Stenting was not possible, due to the membrane position (Fig. 3).
There was no procedural or late complication. The patient recovered very well, and a permanent anticoagulation therapy with warfarin was introduced. The first follow-up was done three months afterwards. Clinically, the patient felt well, there was no evidence of ascites or a peripheral edema. A reduction of collaterals, and only a discrete hepatomegaly was found. An invasive venography was performed, with a planned repeated intervention, if necessary. The result was considered good, with even improved communication throughout the membrane, in comparison with the post-intervention result, so the re-intervention was not performed (Fig. 4). A permanent anticoagulation therapy was recommended to the patient and at the 12-month follow up, she was without any problems.