scholarly journals A rare case of quadrileaflet mitral valve and ostium primum atrial septal defect

Author(s):  
Corrado Fiore ◽  
Tugba Kemaloglu Oz ◽  
Luigi Lombardi ◽  
Rebani Sinani ◽  
Renato Gregorini ◽  
...  

A 60-year-old female was referred to our clinic for evaluation of her rapidly progressive dyspnea, she had no previuos history of heart disease. A murmur was noted on her examination and transthoracic echocardiography was so difficult to be performed due to poor acoustic windows so she was referred to do a transesophageal echocardiography that showed an ostium primum atrial septal defect (ASD) with left to right shunt and a quadrileaflet mitral valve with severe regurgitation. Later on, she underwent surgery with Ostium Primum ASD closure by a patch and double cleft repair by suture after right heart catheterization.

2021 ◽  
Author(s):  
Corrado Fiore ◽  
Tugba Kemaloglu Oz ◽  
Luigi Lombardi ◽  
Rebani Sinani ◽  
Renato Gregorini ◽  
...  

2018 ◽  
Vol 12 (2) ◽  
pp. 91-94
Author(s):  
Md Aslam Hossain ◽  
Dharmendra Joshi ◽  
Mayank Acharya ◽  
Tareq Morshed ◽  
Omar Sadeque Khan ◽  
...  

Mitral regurgitation (MR) associated with secundum Atrial Septal Defect (ASD) is not widely recognized but the association is not unusual. MR has been found in less than 10% of adults with large ASD which is mainly caused by mitral valve prolapse (MVP). We are reporting a case of congenital ostium secundum ASD with MVP associated with MR, review the clinical relevance of this association, and discuss the related literature. Our patient has shown dramatic improvement in symptoms and signs after ASD closure with mitral valve replacement along with improvement in tricuspid regurgitation. More general awareness of this association will further help the surgeon for better management of the patient with this rather unusual combination of lesions.University Heart Journal Vol. 12, No. 2, July 2016; 91-94


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Zarmiga Karunanithi ◽  
Mads Andersen ◽  
Søren Mellemkjær ◽  
Mathias Alstrup ◽  
Farhad Waziri ◽  
...  

Introduction: After atrial septal defect (ASD) correction, pulmonary arterial pressures drop and the right-sided chambers start to remodel. Full normalization may not occur, which can explain the increased morbidity and mortality observed later in life. We described cardiac physiology in adults with a corrected ASD in order to understand the long-term morbidity and mortality. Hypothesis: ASD patients have enlarged right atria and increased pulmonary arterial pressures despite correction. Methods: Participants (percutaneously (n=19) and surgically (n=19) corrected ASD patients and 11 controls of similar age) underwent echocardiography, International Physical Activity Questionnaire, right heart catheterization, cardiopulmonary exercise test, and Holter-monitoring 15-20 years after ASD correction as part of a larger study. Echocardiographic measurements and invasive pressures obtained at rest are reported. Results: Right and left atrial end-systolic volumes, volume index, and filling pressures are higher in corrected ASD patients (particularly the surgically corrected) when compared with controls. Conclusion: ASD patients (particularly surgically corrected) have higher atrial volumes compared with healthy matched controls 15-20 years after correction, while still within the normal range. During systole, the peak atrial pressures are increased in ASD corrected patients, which correlate well with the atrial volumes seen on echocardiography. These findings show that cardiac remodeling has occurred post-correction, but full normalization is not reached. The differences in atrial size and filling pressure in corrected ASD compared to that in controls may be a marker of ASDs leading to myocardial disease and explain why ASD patients experience increased morbidity and mortality later in life.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
B Koell ◽  
S Ludwig ◽  
O Bhadra ◽  
A Gossling ◽  
N Schofer ◽  
...  

Abstract Background Pulmonary hypertension (PH) due to left heart disease is the most common form of PH. Published literature suggests increased perisurgical mortality in patients undergoing surgical repair in the setting of preexisting PH. The data on the impact of preexisting PH on clinical outcomes after percutaneous Mitral Valve Edge-to-Edge Repair (pMVR) is limited to observational studies and rely mostly on echocardiographic data. Purpose The aim of the current study is to evaluate the influence of preexisting PH in patients undergoing pMVR analyzing periprocedural invasive right heart catheterization data. Methods Between September 2008 and July 2018, a total of 911 patients with moderate-to-severe or severe mitral regurgitation (MR) underwent pMVR at our center. This analysis includes 331 patients with a complete data set for pre- and postprocedural right heart catheterization and echocardiographic assessment as well as available follow-up information after the implantation. Patients are divided according to the etiology of PH. The combined primary endpoint consists of all-cause mortality and rehospitalization for heart failure. Furthermore, a sub-analysis is performed for all patients with preexisting post-capillary PH. Patients with post-capillary PH are divided into two groups based on a postprocedural decrease of pulmonary artery wedge pressure (mPAWP) below the threshold of 15mmHg. Univariate and multivariate Cox regression analyses are performed to assess the influence on long-term outcome. Results Of all 331 patients (57.7% [n= 191] male) undergoing pMVR, 195 (62.1%) had functional MR. Median ejection fraction was 40.5% (29.3, 54.0). Patients were followed-up for a maximum of 4.41 years and the median follow-up time was 1.98 years. Preexisting PH (mean pulmonary artery pressure ≥25 mmHg) was found in 236 (71.1%) patients: 49 patients had pre-capillary PH (≤15 mmHg), 187 had post-capillary PH (pcPH; n=183; mPAWP >15 mmHg). In Kaplan-Meier analysis, no statistically significant difference could be found in overall mortality in patients without or with PH, irrespective of etiology (p=0.43). However, in patients suffering from post-capillary PH, patients with a postprocedural reduction of mPAWP below the threshold of 15mmHg showed a significantly lower risk for overall long-term mortality compared to patients without a relevant mPAWP reduction (p=0.018). Multivariate analysis revealed acute postprocedural decrease of mPAWP below 15mmHg in patients with post-capillary PH to have a significant influence on mortality (HR 2.81 [1.35, 5.86]; p=0.006; Figure 1). Conclusion In contrast to previously published findings, the present results were not able to show a significant impact of PH, disregarding its etiology, on outcome. Nevertheless, a postprocedural decrease of mPAWP below 15mmHg in patients with post-capillary PH is associated with a favorable outcome. Figure 1 Funding Acknowledgement Type of funding source: None


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
O Maier ◽  
K Hellhammer ◽  
F Boenner ◽  
S Afzal ◽  
M Spieker ◽  
...  

Abstract Background The rising number of new percutaneous interventions for left-sided heart disease leads to increased occurrence of iatrogenic atrial septal defect (iASD). The percutaneous mitral valve repair (PMVR) for severe, symptomatic mitral regurgitation (MR) also requires intraprocedural puncture of the interatrial septum. In some cases iASD is persisting and becomes haemodynamically relevant with enhanced right heart overload due to significant left-to-right-shunting. Purpose This study aimed to evaluate pre- and periprocedural factors that may favour persistence and haemodynamic relevance of iASD in patients after PMVR. Methods In 2015, 75 consecutive patients with severe MR (age 74.8±10.5y) and following PMVR were enrolled. After 12 months, 57 patients completed their follow up (FU) including clinical conditions, transthoracic echocardiography (TTE), and cardiovascular magnetic resonance (CMR) whenever feasible. We evaluated the impact of comorbidities as well as intraprocedural, haemodynamic and functional characteristics that may favour persistence of iASD by multivariate analysis. Haemodynamic relevance of iASD was defined as right heart overload with predominantly significant enlargement of the right atrium (RA), impairment of right heart function as defined by fractional area shortening (FAC), and ratio of pulmonary to systemic blood flow (Qp/Qs>1) when available. Results 18 out of 57 patients (32%) showed a persistent iASD (+iASD), being associated with a specific combination of comorbidities as well as pre-procedural and periprocedural factors that can be summarised by a multifactorial iASD risk calculator (+iASD vs. -iASD: 6.3±2.9 vs. 3.9±2.7; p=0.0058). 11 iASD (61%) became haemodynamically relevant (+hd iASD) with a significant right heart overload (RA area +hd iASD vs. -hd iASD: baseline 23.1±4.1 vs. 23.2±4.3; FU 30.7±6.3 vs. 20.1±4.6; p<0.0001), reduced RV function (FAC +hd iASD vs. -hd iASD: baseline 41.0±10.3 vs. 29.9±7.2; FU 25.3±7.2 vs. 29.1±13.2; p<0.0156) and left-to-right shunting (Qp/Qs -iASD vs. +hd iASD vs. -hd iASD: 1.0±0.3 vs. 1.7±0.4 vs. 0.8±0.1 L/min; p=0.0011). Conclusion This study shows for the first time, that persistence of iASD can be predicted by pre- and periprocedural factors using a risk calculator that may additionally guide careful follow up imaging and therapeutic action after PMVR to avoid development of progressive heart failure.


2015 ◽  
Vol 72 (6) ◽  
pp. 557-560 ◽  
Author(s):  
Ljupco Mangovski ◽  
Mihajlo Farkic ◽  
Ljiljana Jovovic

Introduction. Transcatheter atrial septal defect (ASD) closure is considered to be a gold standard for patients with the suitable anatomy as compared to cardiac surgery. Reocurrence of ASD after surgical closure is a very rare late complication which can be successfully managed with transcatheter procedure. Case report. We reported a female patient with Noonan syndrome who presented with hemodinamically significant ASD 37 years after the corrective cardiac surgery. Due to numerous comorbidities which included severe kyphoscoliosis, pectus excavatum and multiple surgeries we decided to perform transcatheter closure of ASD. The procedure itself was very challenging due to the patient?s short stature and heart?s orientation in the chest, but was performed successfully. The subsequent follow-up was uneventful and the patient reported improvement in the symptoms. Conclusion. Transcatheter closure of ASD in a patient with Noonan syndrome with the history of surgically corrected ASD can be performed successfully, despite challenging chest anatomy.


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