Challenging Cases of Aortic Prosthesis Dysfunction, the Importance of Multimodality Imaging, a Case Series

ECG-gated multidetector computed tomography (MDCT) is a promising complementary technique for evaluation of cardiac native and prosthetic structures. MDCT is able to provide a broader coverage with faster scan acquisition times that yield higher spatial and temporal resolution for cardiac structures whose quality may be affected by artifacts on ultrasound. We report a case series about the most challenging complications occurring after prosthetic aortic valve implantation in four patients: pannus, paravalvular leak, prosthesis’ misfolding and subaortic membrane reformation. In all the cases, enhanced MDCT using a retrospective protocol provided accurate 3D morphoanatomic information about cardiac and extracardiac structures, improving and speeding up the correct diagnosis and treatment planning. Integrated imaging, in particular with MDCT, is now the present, and it will increasingly be the future in the assessment of cardiac structural pathology.

97 An unusual combination of rare congenital heart defects in a 70 years old lady complaining worsening dyspnea

Methods and results A 70-year-old woman presented to our outpatient clinic complaining of worsening dyspnoea in the last 3 months. She had a medical history of hypertension, diabetes, dyslipidemia, and paroxysmal atrial fibrillation. We performed a comprehensive evaluation starting with a transthoracic echocardiogram that showed a dilatation of right ventricle with normal function, severe pulmonary regurgitation, and moderate tricuspid regurgitation with estimated pulmonary artery systolic pressure of 55 mmHg; the left ventricle had normal dimension and function, with mild aortic and mitral regurgitation, and a subaortic membrane which caused a mild obstruction (maximum gradient 17 mmHg). The cardiac magnetic resonance (CMR) confirmed the enlargement of the right ventricle and of the pulmonary artery trunk (51 mm) and the severity of pulmonary regurgitation (regurgitant fraction of 41%). CMR also clearly showed the VSD just below the subaortic membrane and the left to right shunt with a jet that appeared to proceed straight from the left ventricle through the pulmonary valve (Figure 1A). The estimated Qp/Qs was 1.6 and no intramyocardial late enhancement was present. Pulmonary pressures and pulmonary vascular resistance were normal at the right heart catheterization and the Qp/Qs ratio calculated invasively was 1.45. Considering patient high-risk profile for coronary artery disease, a coronary angiography was also performed showing an abnormal origin of the right coronary artery (RCA) from the mid-portion of the left anterior descending coronary artery (LAD) with two significant stenosis: one involving the bifurcation of RCA and the other the mid-portion of the LAD (Figure 1B). The coronary computed tomography angiography (CCTA) showed a benign course of the RCA anterior to the pulmonary artery towards the auriculoventricular groove (Figure 1C, D). Taking into account all these findings, multidisciplinary heart team decided to perform a cardiac surgery intervention of pulmonary valve and trunk replacement, closure of ventricular septal defect and two coronary bypass grafts on LAD and RCA. Conclusions This case represents a combination of some rare congenital heart abnormalities where multimodality cardiovascular imaging techniques were essential to establish a proper diagnosis and to plan an adequate surgical repair. We hypothesize that the peculiar orientation of the VSD jet may have caused the pulmonary trunk dilatation considering that neither the shunt, nor the pulmonary pressure appear to have been of sufficient magnitude to cause it. Pulmonary ectasia and the damage inflicted by the jet to the cusps of the valve have led to the severe valvular insufficiency. While aortic and tricuspid regurgitation are known to be associated with VSD, to the best of our knowledge this is the first report of pulmonary regurgitation secondary to VSD.

Abstract 17189: Radiation-induced Accelerated Multi-valve Disease In A Patient With Subaortic Membrane

A 69-year-old woman with history of right breast cancer status post lumpectomy and radiation (4.2 cGy) 18 months prior, presented with progressive NYHA class III dyspnea. TTE obtained prior to her cancer diagnosis was notable for normal LV size and wall thickness, a subaortic membrane with systolic mean Doppler gradient of 21 mmHg and a mildly thickened mitral valve without significant stenosis or regurgitation. On presentation, TTE was notable for severely increased concentric LV wall thickness (LV mass index 140 g/m 2 ), preserved ejection fraction, increased RV wall thickness, subaortic stenosis with systolic mean Doppler gradient 35 mmHg, moderate-severe aortic regurgitation, thickened mitral valve with posterior leaflet tethering and anterior leaflet override causing severe mitral regurgitation and moderate mitral stenosis (valve area 1.7 cm 2 by pressure half-time). She underwent a surgical intervention with septal myectomy, subaortic membrane resection, aortic root reconstruction, aortic and mitral valve replacement with bioprostheses. She did well post-operatively and had complete resolution of symptoms following cardiac rehabilitation. Radiation-induced valvular heart disease is linked to the total dose of radiation, use of sequential chemotherapy and time since irradiation, with a typical latency of 10-20 years. However, there is paucity of data regarding the influence of these variables in patients with pre-existing valve disease. This case illustrates dramatic acceleration of underlying aortic and mitral valve pathology in a patient with a subaortic membrane, 18 months after radiation. We also noted development of significant concentric LV hypertrophy that was disproportionate to the degree of obstruction across the sub-aortic membrane. Potential candidates for radiation therapy must be carefully screened for pre-existing valve disease, which should warrant increased vigilance and early screening for progressive valve disease.

Complex Repair of a Completely Fused Nullicuspid Aortic Valve Late in Adolescence

We report a case of an 18-year-old female who presented with severe aortic stenosis and insufficiency, eight years following resection of a subaortic membrane. On echocardiography, she was found to have a completely fused or nullicuspid valve, with three equal sinuses and three commissural fusions. Aortic valve repair included leaflet tricuspidization, three commissurotomies, trileaflet ring annuloplasty, and pericardial leaflet reconstruction. At one year follow-up, the patient is asymptomatic, with stable gradients.

Long-Term Functional Results of Patients Operated for Isolated Discrete Subaortic Stenosis

Background: This study aimed to examine the long-term functional results of patients with isolated discrete subaortic stenosis who underwent subaortic membrane resection and myectomy, using transthoracic M-mode echocardiography and 2D speckle-tracking echocardiography. Methods: Twenty patients operated for isolated discrete subaortic stenosis and 31 controls were included in the study. Patients underwent subaortic membrane resection and myectomy. During the long-term follow up, patients were evaluated with transthoracic M-mode echocardiography and 2D speckle-tracking echocardiography for functional assessment. Results: The mean age at operation and mean duration of follow up was 8.1±5.6 years and 7.2±3.3 years, respectively. Interventricular septal thickness at diastole (0.9±0.1 vs. 0.8±0.1 cm, P = 0.001), ejection time (285.7±26.2 vs. 261.2±24.3 msec, P = 0.001), and aortic strain (15.6±3.7 vs. 10.5±4.0, P < 0.001) were significantly higher in patients. On the other hand, ejection fraction (64.9±6.1 vs. 75.1±5.4 %, P < 0.001), fractional shortening (35.0±5.1 vs. 43.7±5.1, P < 0.001), and corrected velocity circumferential fiber shortening (0.12±0.02 vs. 0.17±0.03, P < 0.001) were significantly lower, when compared with the controls. Longitudinal strain value significantly differed among the groups, with patients having significantly lower strain (18.8±1.8 vs. 20.1±2.1, P = 0.021). Conclusion: In patients operated for isolated discrete subaortic stenosis, aortic gradient seems to continue in the long-term, with the persistence of low longitudinal strain.

Multivalvular destruction as the primary presentation of aggressive infective endocarditis with subaortic valve membrane

Multivalvular destruction may be a clinical manifestation of infective endocarditis (IE), which is a devastating infection of the heart either alone or superimposed with congenital subaortic membrane as in this case report. Here, we report a case of multivavular destruction with severe vegetation presented as a manifestation of infective endocarditis (IE) in a neglected case of 18-year-old male with previous rheumatic heart disease. Transesophageal echocardiography is an important imaging modality for diagnosis of superimposed aortic and heart lesions. Early necessary investigation and correct diagnosis is mandatory to prevent bad complications. doi: https://doi.org/10.12669/pjms.37.2.2798 How to cite this:Alshoabi SA, Aljaber NN, Gameraddin MB, Omer AM. Multivalvular destruction as the primary presentation of aggressive infective endocarditis with subaortic valve membrane. Pak J Med Sci. 2021;37(2):---------. doi: https://doi.org/10.12669/pjms.37.2.2798 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.