Large fenestrated atrial septal occluder to treat an elderly female in her seventh decade

Anil Kumar Singhi; Arindam Pande; Soumya Kanti Mohapatra; Nandini Biswas; Arnab De

doi:10.4103/ACCJ.ACCJ_23_22

CASE REPORT

Year : 2022 | Volume : 4 | Issue : 2 | Page : 92-96

Large fenestrated atrial septal occluder to treat an elderly female in her seventh decade

Anil Kumar Singhi¹, Arindam Pande², Soumya Kanti Mohapatra³, Nandini Biswas⁴, Arnab De⁵
¹ Department of Pediatric and Congenital Heart Disease, Medica Superspecialty Hospital, Kolkata, West Bengal, India
² Department of Cardiology, Medica Superspecialty Hospital, Kolkata, West Bengal, India
³ Department of Pediatric Cardiology, Medica Superspecialty Hospital, Kolkata, West Bengal, India
⁴ Department of Pulmonology, Medica Super Specialty Hospital, Kolkata, West Bengal, India
⁵ Department of Cardiology, Medica Super Specialty Hospital, Kolkata, West Bengal, India

Date of Submission	04-Dec-2022
Date of Decision	10-Jan-2023
Date of Acceptance	22-Jan-2023
Date of Web Publication	02-Mar-2023

Correspondence Address:
Dr. Anil Kumar Singhi
Department of Pediatric and Congenital Heart Disease, Medica Superspecialty Hospital, Mukundapur, Kolkata - 700 099, West Bengal
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ACCJ.ACCJ_23_22

Abstract

Atrial septal defect (ASD) is one of the common congenital heart anomalies presenting in the geriatric group. It presents with symptoms such as progressive effort intolerance, arrhythmias, and palpitations. Sometimes, associated obstructive and restrictive respiratory diseases can contribute significantly to the symptoms masked by cardiac disease. A female in her seventh decade, presenting with dyspnea, had a large ASD with diastolic dysfunction and moderate pulmonary hypertension. She had persistence of symptoms after initial treatment for cardiac elements. On detailed evaluation, she was found to have mixed obstructive and restrictive respiratory disease. She was treated for respiratory elements under expert pulmonology guidance with significant improvement. The ASD was occluded with a custom-made fenestrated 40 mm LifeTech atrial septal occluder in view of diastolic dysfunction and initial pulmonary arterial hypertension. She remained symptomatically better on her short-term follow-up. The index case highlights the importance of detailed evaluation of elderly patients with large ASDs and individualized care to treat.

Keywords: Airway disease, atrial septal defect, device closure, diastolic dysfunction, fenestration, pulmonary hypertension

How to cite this article:
Singhi AK, Pande A, Mohapatra SK, Biswas N, De A. Large fenestrated atrial septal occluder to treat an elderly female in her seventh decade. Ann Clin Cardiol 2022;4:92-6

How to cite this URL:
Singhi AK, Pande A, Mohapatra SK, Biswas N, De A. Large fenestrated atrial septal occluder to treat an elderly female in her seventh decade. Ann Clin Cardiol [serial online] 2022 [cited 2023 May 29];4:92-6. Available from: http://www.onlineacc.org/text.asp?2022/4/2/92/371160

Introduction

Transcatheter closure of atrial septal defect (ASD) is a well-established fact for most of the elderly patients with ostium secundum ASD with suitable anatomy and hemodynamics. Long-standing ASD is known for complications such as pulmonary hypertension (PAH), arrhythmia, and dysfunction. Chronic age-related airway disease can complicate the symptoms of large ASD in elderly patients.^[1] We share the story of an elderly female with large ASD and multiple comorbidities who underwent successful transcatheter treatment.

Case Report

A 62-year-old female presented with exertional dyspnea (New York Heart Association Functional Class III) and exertional palpitation. Her room air oxygen saturation was 90%–92%. On cardiac evaluation, she had a normal first heart sound with wide and fixed split second heart sound. There was 2/6 ejection systolic murmur at the left 2^nd intercostal space. Her chest X-ray showed a cardiothoracic ratio of 0.6 with features of increased pulmonary blood flow. The electrocardiogram showed sinus rhythm with rightward QRS axis and incomplete right bundle branch block. Echocardiography showed a large 32 mm ostium secundum ASD [[Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d, [Figure 1]e, [Figure 1]f, [Figure 1]g, [Figure 1]h and Video 1] [Additional file 1] shunting predominantly left to right with some bidirectionality. The posteroinferior margin of the ASD was small, thin and floppy [[Figure 1]e and Video 1]. The right atrium and right ventricle was aneurysmally dilated. The estimated pulmonary arterial pressure of 64 mmHg by tricuspid regurgitation jet [Figure 1]b suggestive of moderate PAH. She had diastolic dysfunction (Mitral E-E': 12.3) with good left ventricular systolic function (ejection fraction of 61%). She was put on diuretic therapy and beta blockers (bisoprolol). In the follow-up, there was suboptimal improvement in the symptoms. During repeat evaluation, she was found to have bilateral wheeze on auscultation. A pulmonologist consultation was sought. On detailed interrogation, she was found to have a long history of allergic symptoms, sneezing, cough, and wheeze. Her pulmonary function test (PFT) showed forced expiratory volume in one second (FEV1) 0.73 l, 35% of the predicted, forced vital capacity (FVC) was 1.04 l, 41% of the predicted value, and FEV1/FVC ratio was 85% of predicted value. The parameters were suggestive of mixed obstructive and restrictive airway disease [Figure 2]a. High-resolution computed tomography (HRCT) of the chest showed subtle areas of air trapping in both the lung fields and basal atelectasis with evidence of air trapping, obstruction of the small airways, and mosaic attenuation [Figure 3]a and [Figure 3]b. She was treated with metered dose inhalation therapy of formoterol fumarate, tiotropium bromide, budesonide, and oral Monteleucast. Her symptoms significantly improved on respiratory symptoms and oxygen saturation marginally improved to ~94% in 4-week follow-up. The repeat PFT showed FEV1 1.32 l, 64% of the predicted, FVC was 1.68 l, 67% of the predicted value, and FEV1/FVC ratio was 96% of predicted value [Figure 2]b.

Figure 1: Echocardiogram in (a) apical four chamber view showing secundum atrial septal defect (ASD) marked by white arrow. (b) Tricuspid regurgitation gradient. Transesophageal echocardiogram (TEE) in 90-degree view (c and d) showing large ASD left to right shunt (green arrow). (e) TEE in zero degree view showing small thin floppy posterior margin (yellow arrow), (f) Colour Doppler showing left to right shunt and (g) right to left shunt (red arrow). (h) Three dimensional enface TEE view from right atrial side showing the secundum ASD with thin floppy posterior inferior margin (arrow)

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Figure 2: Pulmonary function test at basal state (a) and after appropriate respiratory treatment (b)

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Figure 3: HRCT image shows (a) subtle areas of air trapping in both the lung fields and (b) basal atelectasis with evidence of air trapping and mosaic attenuation. HRCT: High-resolution computed tomography

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The patient was planned for cardiac catheterization for hemodynamic study, followed by fenestrated atrial septal occlusion in view of large ASD with moderate PAH and diastolic dysfunction as found in the echocardiographic assessment along with associated obstructive airway disease. Based on the assessment on transesophageal echocardiography, a 38 mm and a 40 mm atrial septal occluder with custom-made 8 mm fenestration was ordered. The patient and the family were explained and counseled for the procedure and consent obtained. The hemodynamic study showed moderate pulmonary arterial hypertension (pressure of 44/23 with mean of 31 mmHg), mean right atrial pressure of 14 mmHg, right ventricular end diastolic pressure 15 mmHg, and left ventricular end diastolic pressure (LVEDP) of 16 mmHg. The calculated pulmonary blood flow to systemic blood flow ration (Qp/Qs) of 3.1 and pulmonary vascular resistance was 2.46 WU.m2. Coronary artery angiogram showed right dominant coronary artery. There was no evidence of significant atherosclerotic disease. The periprocedure transesophageal echocardiogram showed a thick to thick ASD size of 37 mm. The ASD was a balloon occluded with a 33 mm equalizer balloon (Boston Scientific, Marlborough, MA, USA) over a 0.035 super stiff wire placed across the ASD. Repeat assessment of LVEDP was done after 10 minutes of balloon occlusion and was found to be elevated pressure of 20 mmHg. The ASD was closed with a 40 mm HeartR Atrial Septal occluder (LifeTech Scientific, Shenzhen, PRC) with the custom-made 8-mm fenestration [Figure 4]g. Postdevice deployment, the LVEDP was 18–20 mmHg with mild elevation in the aortic pressure. The oxygen saturation rose to 97%. The pulmonary arterial systolic pressure estimated by TR jet was ~30 mmHg. Detailed transoesophageal echocardiogram showed stable device position with fenestration shunting left to right having mean gradient of 5 mmHg [[Figure 4]a, [Figure 4]b, [Figure 4]c, [Figure 4]d, [Figure 4]e, [Figure 4]f, [Figure 4]g and Video 2] [Additional file 2]. The preprocedure elevated pulmonary artery pressure was considered predominantly flow related. Hence, pulmonary vasodilators were not considered postprocedure. The patient has been doing well on her short-term follow-up of nine months with improvement in the reversible obstructive airway disease.

Figure 4: Transesophageal echocardiogram (TEE) in (a and b) bicaval view and (c) modified apical view (24 degree) showing atrial septal occluder in stable position and custom made fenestration ( arrow) flowing left to right (b). (d) The pulsed Doppler of the fenestration flow showed a mean gradient of 5 mm hg. Transesophageal three dimensional enface view from right atrial side showing the (e) atrial septal occluder with fenestration (arrow) and (f) 3D color flow (green arrow). (g) The custom made fenestration (red arrow) of 40 mm Lifetech atrial septal occluder is shown

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Discussion

Transcatheter device closure is the treatment of choice for suitable secundum ASDs in various age groups. The long-standing left-to-right shunt in ASD is associated with arrhythmias, myocardial stiffness, and pulmonary arterial hypertension. Presence of comorbidities like obstructive pulmonary disease, ischemic heart diseases, and hypertension influences the clinical scenario and makes treatment more challenging.^[1] In the late age, left ventricular myocardium develops age-related elastic stiffness and reduced diastolic compliance. Prolonged left-to-right shunt in the patients with ASD influences the interventricular septal configuration. Bowing of the interventricular septum is seen toward the left ventricle causing under-filling of the left ventricle. Device closure leads to a significant elevation of LVEDP by unmasking of ventricular restriction. The phenomenon of significant elevation of LVEDP postdevice closure is reported in 2%–23.6% of the older patients with ASD in various studies.^[2],[3] The elevated LVEDP after complete closure of ASD may lead to acute hemodynamic change causing acute pulmonary edema.^[4],[5] The latent left ventricular diastolic dysfunction can be assessed during catheterization by measuring the LVEDP at the baseline and then 15 min after occluding the ASD by an appropriate balloon or with the device itself.^[6] If pressure increases by 5 mmHg after the occlusion, the device requires fenestration with a continuation of the heart failure medication.^[2],[7]

Mild-to-moderate PAH is a well-known in long standing ASD in the elderly population. Pulmonary arterial systolic pressure more than 50 mmHg is reported by Cherian et al. in 17% of their cohort of older ASD patients.^[8] Usually, the pulmonary pressure decreases or remains steady in mild levels after the closure. The PAH may persist even after closure of the ASD. There can be delayed PAH after ASD occlusion. The patients with ASD and significant left-to-right shunt along with persistently elevated pulmonary pressure after balloon occlusion can be closed with fenestrated ASD device.^[1]

The elderly patients with large ASD can have age-related changes in the airways leading to symptoms of Chronic obstructive pulmonary disease (COPD). The obstructive airway disease is a known contributor to the elevated pulmonary artery pressure. The symptoms of the COPD are frequently masked by the symptoms of heart disease. Significant reduction in the COPD symptoms was reported in the literature in the majority of the patients who had initially COPD symptoms. Acute episodes of exacerbation of airway disease may show signs of right ventricular failure which can add to the existing symptom of long standing ASD. Nassif et al. reported a high prevalence of airway hyperresponsiveness in an adult cohort of unrepaired ASD patients and linked it with the asthma-like symptoms. The symptoms potentially required pulmonary inhalant use, both before and long after closure. They suggested attention to symptoms and pulmonary function during clinical follow-up of adult ASD patients, both before and long after closure.^[9] This has the double benefit of symptomatic improvement and reduction in the PAH.^[10]

Detailed assessment of comorbidities and associated clinical conditions like PAH and cardiac functions of older patients with ASD is essential for successful treatment. There can be multiple issues in the same patient. Chronic obstructive lung can add to the symptoms which need to be evaluated in suspected patients. The associated clinical conditions with ASD like right heart dysfunction and left ventricular diastolic dysfunction can be treated along with COPD therapy for symptomatic improvement. Significant diastolic dysfunction and PAH in the backdrop significant left-to-right shunt requires fenestrated device closure of ASD for a favorable pop-off. The recommended criteria for the fenestration is either elevated pulmonary artery pressure in the setting of significantly increased pulmonary blood flow or elevated LVEDP as seen in balloon occlusion of the ASD. The LifeTech custom-made fenestrated atrial septal occluder has 5 mm fenestration in 22 and 24 mm occluder, 6 mm fenestration in 26–30 mm occluder, and 8 mm fenestration in 32, 34 mm occluder. The fenestration size was decided by the company on testing the stability and integrity of the structure. Larger size fenestrated occluder may have to be tailor made to suit the requirement of the patient.

Conclusion

Transcatheter fenestrated closure of ASD can be done for elderly patients associated with multiple comorbidities like PAH, diastolic dysfunction, and associated COPD. There has to be an individualized approach for fenestrated closure of ASD based on hemodynamic status and comorbidities. Detailed evaluation and treatment of an index patient in a multimodality team approach is the key for success.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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