Atrial Septal Defect and Platypnea-Orthodeoxia Syndrome: Usefulness of the Balloon Occlusion Test

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J INVASIVE CARDIOL 2024. doi:10.25270/jic/24.00153. Epub May 23, 2024.

A 37-year-old man was referred to our medical center with a diagnosis of Eisenmenger syndrome due to an atrial septal defect (ASD). At admission he had central cyanosis, acrocyanosis, and progressive effort dyspnea; his symptoms improved during lying position. Physical examination revealed cyanosis, clubbing, a pulmonic systolic murmur II/IV, and SO2 between 50% and 60% on standing position and between 84% and 88% on lying position. Blood tests showed erythrocytosis (hemoglobin 20g/dL, hematocrit 60%). Transthoracic (TTE) and transesophageal echocardiography (TOE) showed a bidirectional with predominantly right to left shunt ASD of 24 mm with a spiral septum and proper rims for percutaneous closure, and normal pulmonary pressure (Figure 1A and B, Videos 1 and 2). Right heart hemodynamics demonstrated a systolic pulmonary artery pressure of 17 mm Hg, a right atrium pressure of 4 mm Hg, and a QP:QS og 1.8:1.

A balloon occlusion test (BOT) was performed using a 35-mm Occlutech sizing balloon (Figure 1C). Using the stop-flow technique, the ASD was measured at 25 mm. Pulmonary and systemic pressure showed no changes after 15 minutes. The aorta blood gas analysis improved from 88% to 97% at room air. We decided first to close the ASD with a 27-mm Figulla Flex II Septal Occluder, however, due to the spiral septum it was impossible to attach it to both sides of the septum. Finally, the ASD was sealed by deploying a 33-mm occluder (Figure 1D).  Six-months postoperative, the patient is asymptomatic and has stopped using oxygen, oxygen saturation is on 95% without supplementary oxygen, hemoglobin is on 13.4g/dL, and TTE shows no residual shunt (Figure 1E and F).

Platypnea-orthodeoxia syndrome (POS) is a rare condition that in most cases is related to a large patent foramen ovale.1, 2 This is a case where an ASD with spiral septum promoted a large right to left shunt. We demonstrate the usefulness and feasibility of the BOT3 for the closure decision with excellent outcomes and reversal of cyanosis, erytrhocytosis, and clubbing (Figure 1G and H).

Affiliations and Disclosures

From the Mexican Social Security Institute Bajío Center UMAE T1, León Guanajuato, México.

Acknowledgments:  Enrique Alfredo Bernal Ruíz, MD (participating investigator) served as scientific advisor. Fidel Ortiz Alcalá, MD (participating investigator) critically reviewed the study proposal. Cristian Adrián Villar Valencia, MD (participating investigator) provided and cared for study patients.

Disclosures: The authors report no financial relationships or conflicts of interest regarding the content herein.

Consent statement: Patient informed consent was obtained prior to publishing in accordance with COPE guidelines.

Address for correspondence: Marco Alejandro Solórzano Vázquez, MD, Circunvalación poniente, No. 309-828 Int 401, Colonia Valle del Campestre, León Guanajuato 37150, México. Email: dr.solorzano.alejandro.ci@gmail.com

References

1. Henkin S, Negrotto S, Pollak PM, Cullen MW, O'Cochlain DF, Wright RS. Platypnea-orthodeoxia syndrome: diagnostic challenge and the importance of heightened clinical suspicion. Tex Heart Inst J. 2015;42(5):498-501. doi: 10.14503/THIJ-14-4596
2. Salas-Pacheco JL. Mecanismos del síndrome de platipnea-ortodesoxia [Mechanisms of platypnea-orthodeoxia syndrome]. Arch Cardiol Mex. 2022;92(2):274-282. doi: 10.24875/ACM.21000171
3. Shah AH, Osten M, Leventhal A, et al. Percutaneous intervention to treat platypnea-orthodeoxia syndrome: the toronto experience. JACC Cardiovasc Interv. 2016;9(18):1928-1938. doi: 10.1016/j.jcin.2016.07.003