(II-1A.4d) 62-year-old female with Ostium Secundum Atrial Septal Defect (ASD t. II) and coexisting pulmonary hypertension Stepniewski J., Podolec M., Komar M., Wójtowicz M., Tyrka A., Nowacka M., Kopeć G., Rubiś P., Tomkiewicz-Pająk L.,
Experts: Sadowski J., Kapelak B., Podolec P.

Ostium Secundum Atrial Septal Defect (ASD II) is the most commonly diagnosed type of ASDs. It accounts for about 75% of all ASD cases, representing approximately 7% of all congenital cardiac defects and 30-40% of all congenital heart disease in patients older than 40 years. It is characterized by a defect in the interatrial septum allowing pulmonary venous return to pass directly from the left atrium to the right atrium. The condition can be left undiagnosed for many years until developing signs of exercise intolerance with dyspnea and fatigue on exertion. The most frequently observed complications of this anomaly are supraventricular arrhythmias including atrial flutter and atrial fibrillation. Pulmonary hypertension (PH) as a result of increased pulmonary blood flow is observed relatively rare. The estimated prevalence of PH in patients with ASD is about 10%. The authors present a case of patient with ASD II and coexisting pulmonary hypertension.

Case description
A 62-year-old Caucasian female with Ostium Secundum Atrial Septal Defect diagnosed in transesophageal echocardiogram (TEE) in 2004, currently admitted to the hospital with symptoms of heart failure in class III by NYHA (New York Heart Association) including exercise intolerance, fatigue and dyspnea on exertion.
She did not give consent for surgical repair of the defect at the time of diagnosis in 2004.
By the time of present admission, she has recurrently been hospitalized due to heart failure exacerbations. She has a history of arterial hypertension, obesity (BMI- 35.6 kg/m2), lipid abnormalities, gall stones and thyroidectomy due to nodular goiter in 2008.
Her oral pharmacotherapy includes angiotensin-converting enzyme inhibitor (ACEI) 10mg once a day (q.d.), calcium channel blocker 5mg q.d., indapamide q.d., spironolactone 50mg q.d., ASA 75mg q.d., atorvastatin 20mg q.d., levothyroxine 100ug q.d.
On admission, she is hemodynamically stable with no signs of pulmonary oedema or peripheral tissue swelling. Her blood pressure is 180/115mmHg and heart rate 80 beats/min. ECG- sinus rhythm of 75 beats/min, left axis deviation, signs of right bundle branch block (RBBB). The blood analysis does not reveal any abnormalities apart from increased level of total cholesterol (TC) of 5.49mmol/l. Her exercise capacity is considered to be poor after terminating cardiopulmonary exercise test (CPET) in the 1st minute due to severe dyspnea.
Transthoracic (TTE) and transesophageal echocardiography examination (TEE) is performed in order to evaluate heart function and morphology of the septal defect. TTE reveals signs of right heart volume overload with right ventricular (RV) dilation of 43.6/42 mm, right atrial (RA) dilation of 52.6/56.9mm, pulmonary hypertension (PH) with estimated right ventricular systolic pressure (RVSP) of 50mmHg and pulmonary artery (PA) diameter of 44mm, right ventricular diastolic dysfunction with normal left ventricular (LV) function of 65% of ejection fraction (EF) and left-to-right shunt of 1.56 Qp/Qs ratio. TEE shows ASD with the maximal size of 22mm with no aortal rim and little mitral rim, significant left-to-right shunt and flaccid atrial septum. Right heart catheterization reveals pulmonary hypertension with mean PA pressure (PAP) of 44 mmHg decreasing to 36 mmHg after nitric oxide inhalation (NO) and pulmonary vascular resistance (PVR) of 640 ARU (8 Wood Units [WU]) decreasing after NO inhalation to 357 ARU (4.5 WU). Systemic vascular resistance (SVR) is 1775 ARU decreasing to 1653 ARU after NO inhalation. No changes in the coronary arteries are detected in coronary angiography. Lung scintigraphy shows numerous, subsegmental perfusion deficits, corresponding to vascular changes in the course of pulmonary hypertension.

Following the European Society of Cardiology guidelines for intervention in atrial septal defect cases patients with pulmonary vascular resistance (PVR) equal or above 5 Wood Units but with PVR less than two third of systemic vascular resistance or pulmonary artery pressure less than 2/3 of systemic pressure and left-to-right shunt of more than 1.5 Qp/Qs ratio may be considered for intervention.

1. Vogel M, Berger F, Kramer A, Alexi-Meshkishvili V, Lange PE; Heart. 1999;82(1):30 Incidence of secondary pulmonary hypertension in adults with atrial septal or sinus venosus defects.
2. ESC Guidelines for the management of grown-up congenital heart disease. European Heart Journal (2010) 31, 2915–2957.

Experts’ comments:
1. Podolec Piotr: ASD closure is recommended. Unless unsuitable morphology of the defect (no mitral edge), percutaneous approach would be the procedure of choice.
2. Jerzy Sadowski, Boguslaw Kapelak: Procedural risk related to obesity, pulmonary hypertension is high. Additional problem is the lack of patient cooperation during the treatment process. The patient should be well informed about the increased risk.

Surgical ASD closure may be considered at this point. Final decision belongs to the patient. Information considering increased procedural risk must be clearly explained to the patient. Informed consent must be taken prior to the procedure.

Stepniewski J.1, Podolec M.1, Komar M.1, Wójtowicz M.1, Tyrka A.1, Nowacka M.1, Kopeć G.1, Rubiś P.1, Tomkiewicz-Pająk L.1

Sadowski J.2, Kapelak B.2, Podolec P.1

1 Department of Cardiac and Vascular Diseases, John Paul II Hospital, Kraków, Poland
2 Department of Cardiac, Vessels Surgery and Transplantology, Jagiellonian University College of Medicine, John Paul II Hospital, Krakow, Poland


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