Combined transcatheter treatment of severe mitral regurgitation and secundum atrial septal defect in an inoperable patient: a case report

Background Chronic mitral regurgitation is one of the most common valvular heart diseases and is associated with poor outcome. Although other structural diseases are regularly seen in such patients, concomitant atrial septal defects remain a rarity in the elderly. Case summary We report a case of an 82-year old woman with progressive right-sided heart failure due to mitral regurgitation and an atrial septal defect of secundum type, despite optimal medical therapy. Combined transcatheter mitral valve repair by utilizing a separate transseptal puncture and atrial septal defect closure was performed resulting in amelioration of symptoms. Discussion Procedural planning for simultaneous transcatheter therapies of coupled structural heart disease entities remains complex. Our case illustrates feasibility of percutaneous edge-to-edge mitral valve repair and consecutive closure of a large secundum atrial septal defect. Different options of accessing the left atrium should be discussed on an individual basis, while additional atrial septal defect closure may be beneficial in terms of right ventricular function and symptoms of right heart failure.

The Combination of Oral PDE5-Inhibitor (Sildenafil) And Oral Prostacy- clin Analogue (Beraphrost) Therapy for Increasing Quality of Life in Adults with Pulmonary Arterial Hypertension Related to Uncorrected Secundum Atrial Septal Defect

Background : Sildenafil, an oral phosphodiesterase type-5 inhibitor, has vasodilatory effects through a cyclic guanosine 3,5-monophosphate–dependent mechanism, whereas beraprost, an oral prostacyclin analog, induces vasorelaxation through a cAMP-dependent mechanism. This combination has often used but there was little detailed study on it Objectives : To investigate whether the combination of oral sildenafil and beraprost is superior to sildenafil alone in in adult patients with Pulmonary Arterial Hypertention (PAH) related uncorrected secundum Atrial Septal Defect (ASD). Methods : Patients with secundum ASD who developed PAH divided into two group. Group A received oral sildenafil 3x40 mg and oral beraphrost 3x20 mcg. Group B received oral sildenafil only 3x40 mg in a 12-week. Health-related quality of life (HRQoL) was recorded by patients using the Medical Outcomes Study 36-item short form (SF-36) questionnaires at baseline and after 12 of therapy. Therapy adherence was achieved through a series of phone calls and a four-weekly hospital visit. Every normal follow-up appointment included an examina- tion of side effects and a dosage modification based on the clinical situation Results: We didn’t found any significant of proportion different in cofounding factor between groups. Compared with Group B, Group A had better functional capacity, limitation to physical health, energy fatigue, pain, and health change (P=0.00, P=0.03, P=0.044, P=0.026, P=0.008, respectively). Conclusion: Combination between oral sildenafil therapy 40 mg three times per day and beraphrost 20 mcg two times per day significantly increase the HRQoL in PAH patients in uncorrected secundum ASD compared sildena- fil alone

Diameter and shunt act as an alleviator of pulmonary hypertension: an echocardiography assessment of patients with ostium secundum atrial septal defect

Background Ostium-secundum atrial septal defect (OS-ASD) increases pulmonary arterial systolic pressure (PSAP) and eventually leads to pulmonary hypertension (PH). Nevertheless, whether the characteristics of OS-ASD could serve as an alleviator within chronic-volume overload in the right atrium (RA) is unclear. Aims To evaluate a potential interaction of OS-ASD diameter and the shunt (Qp/Qs ratio) on PSAP values in patients with secondary PH. Methods We designed a cross-sectional study of 63 adult patients with OS-ASD. A conventional transthoracic echocardiography was performed using Vivid 9X-clear equipment (GE Vingmed Ultrasound, Horten, Norway) to evaluate the OS-ASD diameter and the Qp/Qs ratio. The PSAP was calculated by tricuspid regurgitation velocity with continuous-wave Doppler in the apical four-chamber view. Polynomic adjusted correlations were performed between the RA volume and the PSAP; we evaluated a double and triple interaction-effect of the OS-ASD diameter and Qp/Qs ratio, respectively adjusted for age, sex and body surface area. Results In our study population, 78% (n=49) were female, with a mean age of 41.8 (±15) years. 44% (n=28) with mild-PH, 38% (n=24) with moderate-PH and 17% (n=11) with severe-PH. Mean echocardiographic parameters were: RA volume 58.8 (±26.4) mL, PSAP of 55.9 (±20.7) mmHg, OS-ASD diameter 30.1 (±7.8) mm and Qp/Qs 2.9 (±0.9). We observed a cubic relationship between RA volume and PSAP (r=0.354, p=0.006; Figure 1A). ASD-Diameter had a negative interaction effect (β=−0.0231 95% CI: −0.043 to −0.003, p=0.03) of the relationship between RA volume and PSAP (Figure 1B). Furthermore, the addition of Qp/Qs ratio displayed a positive triple interaction effect (β=0.021 95% CI: 0.001–0.043, p=0.048) on the previously mentioned variables (Figure 1C) after adjusting for covariates. Conclusions The increase in OS-ASD diameter and shunt decreases PSAP values. Our results could indicate that OS-ASD could potentially ameliorate chronic-volume overload in patients with secondary PH. FUNDunding Acknowledgement Type of funding sources: None. Figure 1

Fraser Syndrome With Megalencephaly: A Rare Association

An outborn term neonate was referred at 12 h of life with multiple congenital anomalies. A baby was born out of consanguineous marriage to a mother with 2 previous abortions. Clinical examination showed features such as bilateral cryptophthalmos, syndactyly involving all limbs, and clitoromegaly suggestive of Fraser syndrome. Ultrasound of the abdomen showed unilateral renal hypoplasia. Echocardiography showed ostium secundum atrial septal defect, small ventricular septal defect, and patent ductus arteriosus. Magnetic resonance imaging of the brain revealed megalencephaly with polygyria. Further evaluation and surgical treatment of cryptophthalmos were advised but could not be done because of socioeconomic constraint, and the neonate was discharged and lost to follow-up. The occurrence of cerebral malformations in Fraser syndrome is highly variable and not very well described in the literature. To the best of our knowledge, association of megalencephaly with this syndrome is being reported for the first time. We also intend to educate the physicians about the antenatal clues associated with this rare syndrome, which could promote antenatal diagnosis and thereby modify the outcome.

Congenital Nephrotic Syndrome and the Heart: Lest We Forget!

We report a 2-month-old girl, presenting with anasarca in the early infancy, and was diagnosed to have congenital nephrotic syndrome. In view of a systolic murmur, echocardiography was done which revealed ostium secundum atrial septal defect and branch pulmonary artery stenosis. Genetic analysis was suggestive of single base pair duplication, resulting into frame shift mutation of NPHS1 (nephrin) gene.

Stroke in patients with secundum atrial septal defect and sequelae after transcatheter closure

ObjectiveTo evaluate the frequency of and risk factors for stroke as a presenting feature in adult patients with secundum atrial septal defect (ASD); rates of post-closure atrial fibrillation (AF) and stroke were also assessed.MethodsWe retrospectively reviewed adult patients who presented with an ASD between 2002 and 2018, excluding those with known atrial arrhythmias. Risk factors for stroke were identified using multivariable logistic regression. Post-closure stroke was evaluated using survival analysis stratified by the presence of post-procedure AF.ResultsOf 346 patients with ASD (median age 44 years), 34 (10%) presented with a history of stroke. Independent risk factors included elevated body mass index over 25 (OR: 18.2; 95% CI: 4.0 to 82.2; p<0.001), smoking (OR: 9.5; 95% CI: 3.8 to 23.9; p<0.001) and a prominent Eustachian valve (OR: 9.2; 95% CI: 3.4 to 25.2; p<0.001). A scoring system based on these three parameters provided robust stroke risk stratification. During a median follow-up of 12 months after closure, 12 patients (4%) experienced AF and 4 patients (1%) had a new stroke. AF was highly associated with development of stroke post-closure (p<0.001).ConclusionsIn this study population, the incidence of stroke prior to ASD closure among patients without atrial arrhythmias was 10%. Risk factors included obesity, smoking and prominent Eustachian valve anatomy. Lifestyle changes should be recommended for at-risk patients, and it may be reasonable to consider ASD closure in the absence of haemodynamic indications in patients at increased risk of stroke.