In January 2009, a 45-year-old man with a history of 2 aortic valve replacements was referred to our institution with progressive shortness of breath and a severe paravalvular leak. The patient had undergone aortic valve replacement with a mechanical valve at age 20 years for what he described as rheumatic disease. At age 43 years, he underwent repeat aortic valve replacement with a St. Jude mechanical valve after experiencing endocarditis and an embolic cerebrovascular accident with residual right-sided hemiparesis. During the 6 months after that surgery, he developed 2 subdural hematomas that required surgical evacuation and drainage in the setting of a supratherapeutic international normalized ratio due to his need for warfarin therapy. During the next 18 months, he continued to experience dyspnea from worsening heart failure that was caused by the valve disease. Due to his comorbidities, he was considered to be a high-risk surgical candidate for a 3rd aortic valve replacement, so he was referred to our institution for possible percutaneous closure of the paravalvular leak.
At presentation, the patient was cachectic and afebrile, with a blood pressure of 98/55 mmHg, a heart rate of 93 beats/min, a respiratory rate of 16 breaths/min, and an oxygen saturation of 100% on room air. He had mildly elevated jugular venous pressure, clear lungs on auscultation, and a high-pitched decrescendo murmur at the left sternal border in early diastole. Laboratory analysis showed no evidence of hemolysis, and 4 sets of blood cultures grew no bacteria. A transesophageal echocardiogram (TEE) revealed a cavity (1.2 × 1 cm at largest diameter) below the aortic valve ring with severe paravalvular leakage draining anteriorly into the left ventricle ().
Fig. 1 Transesophageal echocardiography shows a severe paravalvular leak, which communicates with a cavity (1.2 × 1 cm in largest diameter) below the aortic valve ring anteriorly and drains into the left ventricle (LV). A) Two-dimensional image (more ...)
The patient was started on diuretic therapy and prophylactic antibiotics. After a multidisciplinary discussion that included cardiologists, cardiothoracic surgeons, primary-care physicians, and the patient, it was concluded that reoperation posed an excessive risk (Society of Thoracic Surgeons operative risk score, >30%).8
The patient agreed to undergo percutaneous closure of the paravalvular leak. The procedure was performed while he was under general anesthesia. Fluoroscopy and real-time 2-dimensional (2D) and 3-dimensional (3D) TEE were used along with right-heart catheterization that revealed normal right- and left-sided filling pressures and normal cardiac output—positive results of the diuretic therapy. Coronary angiography showed no significant coronary artery disease; however, the native left anterior descending coronary artery (LAD) was small, and a larger, anomalous LAD branch arose from the proximal right coronary artery and did not take an intra-arterial course (). Aortography confirmed grade 4 aortic regurgitation through a paravalvular leak.
Fig. 2 Coronary angiograms (left anterior oblique views) show no significant coronary artery disease. Also shown are A) the bifurcation of the left main coronary artery into the left circumflex coronary artery (LCx) and the small, native left anterior (more ...)
Intraprocedural TEE also indicated paravalvular leakage between the aorta and the left ventricular outflow tract through a cavity in the right anterior cusp of the aortic valve. The cavity was crossed with use of a 6F multipurpose catheter and a 0.035-in Terumo Glidewire® (Terumo Medical Corporation; Somerset, NJ). Selective angiography through the defect confirmed the presence of a complex paravalvular fistula to a large cavity below the valve ring, with drainage into the left ventricle (). The use of real-time 2D and 3D TEE along with standard angiography enabled us to estimate the size of the paravalvular leak and thus to attempt closure with a 10-mm AMPLATZER® Vascular Plug II (AGA Medical Corporation; Plymouth, Minn).
Fig. 3 Selective angiography through the defect shows a complex paravalvular fistula to an inferior cavity below the aortic valve ring. A 6F multipurpose catheter has entered the cavity.
The plug was positioned. However, before its deployment, repeat angiography and TEE showed a substantial residual leak adjacent to the plug, suggesting that the plug was too small. It was removed without difficulty. A 7F delivery sheath was inserted, and, due to its more favorable design with 2 concentric discs, a 6-mm AMPLATZER® Muscular VSD Occluder (AGA Medical) was deployed accurately and easily. However, repeat angiography and TEE revealed a residual leak ().
Fig. 4 Two-dimensional transesophageal echocardiogram shows a residual paravalvular leak after deployment of the 1st closure device (CD), a 6-mm AMPLATZER® Muscular VSD Occluder. A high-velocity regurgitant jet is seen superiorly.
We decided to cross the area adjacent to the Amplatzer VSD occluder to deploy a 2nd closure device. The course of the Glidewire was verified by means of 3D TEE. The crossing was challenging, because the Glidewire repeatedly prolapsed into the subaortic cavity. A Quick-Cross® 0.035-in guiding catheter (The Spectranetics Corporation; Colorado Springs, Colo) was successfully positioned over the Glidewire. The Glidewire was then exchanged for a stiffer 0.035-in Rosen wire, and the delivery of a 6F multipurpose catheter enabled the placement of a 10-mm Amplatzer Vascular Plug II adjacent to the Amplatzer VSD occluder. Echocardiography showed excellent device positioning and complete closure of the paravalvular leak (). Angiography showed excellent positioning, no interference with the adjacent valve leaflets, and no evidence of regurgitation. Coronary angiography revealed no impingement on the coronary arteries.
Fig. 5 Two-dimensional transesophageal echocardiogram (at 115°) shows the closure of the paravalvular leak after a 10-mm AMPLATZER® Vascular Plug II (CD2) was deployed near the 6-mm AMPLATZER Muscular VSD Occluder (CD1).
The patient was treated with heparin throughout the procedure to prevent bleeding. Afterwards, he received ongoing warfarin therapy and was discharged from the hospital after 5 days with no sequelae. A follow-up cardiac computed tomogram was obtained to define the anomalous LAD and to obtain a 3D image showing the relationship between the 2 closure devices. The anomalous LAD coursed in front of the pulmonary artery with no impingement, and no reconstructive surgery was thought to be necessary. The patient was scheduled to return to the clinic in 6 to 8 weeks for repeat echocardiography, and for evaluations at 3, 6, and 12 months thereafter; however, he attended no appointments and was lost to follow-up.