Atrial Septostomy to Treat Stiff Left Atrium Syndrome

HomeCirculation: Heart FailureVol. 10, No. 7Atrial Septostomy to Treat Stiff Left Atrium Syndrome Free AccessCase ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessCase ReportPDF/EPUBAtrial Septostomy to Treat Stiff Left Atrium Syndrome Pranav Chandrashekar, MBBS, Jae Yoon Park, MD, Mohammad A. Al-Hijji, MD, Yogesh N.V. Reddy, MBBS, Chad J. Zack, MD, Guy S. Reeder, MD, Robert F. Rea, MD and Barry A. Borlaug, MD Pranav ChandrashekarPranav Chandrashekar From the Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN. , Jae Yoon ParkJae Yoon Park From the Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN. , Mohammad A. Al-HijjiMohammad A. Al-Hijji From the Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN. , Yogesh N.V. ReddyYogesh N.V. Reddy From the Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN. , Chad J. ZackChad J. Zack From the Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN. , Guy S. ReederGuy S. Reeder From the Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN. , Robert F. ReaRobert F. Rea From the Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN. and Barry A. BorlaugBarry A. Borlaug From the Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN. Originally published10 Jul 2017https://doi.org/10.1161/CIRCHEARTFAILURE.117.004160Circulation: Heart Failure. 2017;10:e004160IntroductionRadiofrequency ablation is an effective therapy to restore sinus rhythm in patients with symptomatic atrial fibrillation. Approximately 1.4% of patients undergoing ablation develop unexplained dyspnea because of isolated left atrial (LA) hypertension.1,2 This condition has been termed the stiff LA syndrome. Ideal treatment strategies for stiff LA syndrome remain unclear.CaseA 71-year-old man presented with dyspnea. He had a history of paroxysmal atrial fibrillation/flutter treated with 3 ablation procedures in the preceding 9 years. Physical examination revealed a rapid, irregular heart rate at 173 and jugular venous distention to 10 cm. ECG demonstrated atrial tachycardia. Echocardiogram demonstrated left ventricular ejection fraction of 50%, biatrial enlargement, mild mitral regurgitation, mild right ventricular enlargement, mild–moderate right ventricular systolic dysfunction, and an estimated right ventricular systolic pressure of 43 mm Hg.He was admitted and rate controlled. However, he continued to complain of severe dyspnea after dismissal, despite diuresis. A cardiopulmonary exercise test demonstrated peak oxygen consumption (VO2) of 10.9 mL/kg/min (43% predicted), suggesting severe cardiac limitation. Chest computed tomography demonstrated normal pulmonary vein anatomy without stenosis.Cardiac catheterization was performed with transseptal puncture (Figure 1). Right atrial pressure was mildly elevated (10 mm Hg). The mean pulmonary arterial pressure was moderately elevated (39 mm Hg) with mildly increased pulmonary vascular resistance (3.3 WU). LA pressure at mid C wave and LV end diastolic pressures were only mildly elevated (16 mm Hg), but there was a giant V wave to 39 mm Hg (Figure 1A). There was no gradient between the right- or left-sided pulmonary artery wedge pressures and LA pressure. Cardiac index was normal (2.7 L/min/m2). With the dramatically elevated LA V wave, near-normal LV end diastolic pressure, and absence of significant mitral regurgitation, the diagnosis of stiff LA syndrome was made.1Download figureDownload PowerPointFigure 1. Direct left atrial hemodynamic pressure tracings obtained after transseptal puncture. (A) Prior to septostomy, demonstrating giant V waves to 39 mm Hg (red arrow). (B) Immediately after septostomy, demonstrating significantly decreased V wave to 20 mm Hg. (C) One-year postprocedure tracing showing sustained decrease in V wave pressure.Given the lack of response to medical therapy, the patient was presented with the option of palliative atrial septostomy. The patient agreed to the procedure and underwent atrial balloon septostomy using a 15×40 mm Tyshak pediatric valvuloplasty balloon (NuMED, Inc., Hopkinton, NY). Intracardiac echocardiography demonstrated a 1.5×0.7 cm defect after dilatation (Figure 2). After the procedure, the LA V wave decreased by 50% to 20 mm Hg (Figure 1B).Download figureDownload PowerPointFigure 2. Post-septostomy transthoracic echocardiogram demonstrates an intera trial shunt with blood flow from left to right.The patient reported immediate improvement and was discharged from the hospital the following day. Four months later, he continued to report symptomatic improvement. Repeat cardiopulmonary exercise testing study demonstrated an increase in peak VO2 to 13.9 mL/kg/min (58% predicted). One year after discharge, symptoms remained minimal. The LA V wave pressure remained stable (18 mm Hg) at repeat catheterization performed as part of a percutaneous LA appendage occlusion device implantation (Figure 1C). Echocardiography has not revealed right ventricular enlargement or worsening pulmonary hypertension, with persistent left to right shunt and interatrial pressure gradient of 7 mm Hg.DiscussionIn patients with prior LA ablation presenting with dyspnea or pulmonary hypertension, the differential diagnosis includes heart failure with preserved ejection fraction, pulmonary vein stenosis, mitral regurgitation, or stiff LA syndrome. Cardiac computed tomography was normal, and no pressure gradient was observed between the PA wedge pressure and the LA pressure, ruling out pulmonary vein stenosis. Heart failure with preserved ejection fraction is the most common cause of dyspnea in this population, but the markedly elevated V wave that was out of proportion to the mildly elevated end-diastolic pressure makes that diagnosis less likely. Significant mitral regurgitation was excluded by echocardiography and physical examination. Large V waves on LA pressure tracings in the absence of significant mitral valve disease in this setting is consistent with severely depressed LA compliance because of stiff LA syndrome.1Treatment for stiff LA syndrome is challenging because LV filling pressures are normal or near normal, yet pulmonary artery and venous pressures are elevated owing to the increased V wave amplitude (Figure 1). Diuretics are used but often prove unsuccessful in improving symptoms, as in this patient.1The concept for this approach comes from the observation that patients with mitral stenosis and an atrial septal defect fair better than those with isolated mitral stenosis. We hypothesized that a similar beneficial effect might be achieved in our patient by creation of an interatrial communication. LA pressure is related to the volume of blood in the LA, as well as its operant stiffness, which increases at higher LA volumes (Figure 3). Atrial septostomy enables LA unloading via left to right shunt when LA pressure is its highest, corresponding to the points on the LA compliance curve that are steepest, as at the peak of the V wave (Figure 3).Download figureDownload PowerPointFigure 3. Hypothetical left atrial (LA) pressure–volume loops in this patient prior to septostomy (A) and after septostomy (B). The LA diastolic pressure–volume relationship is steep at higher volumes; thus, a small increase in LA volume in this range leads to a large increase in LA pressure with increase in the peak of the V wave. With the shunt induced by septostomy, LA volume shifts down to the more compliant range in the pressure–volume relationship, where V wave pressure amplitude is decreased.Abnormalities in LA structure and function are also common in people with heart failure with preserved ejection fraction.3 Recent studies testing novel devices enabling left-to-right interatrial shunting in this cohort have yielded promising results.4 Because patients with the stiff LA syndrome display the most profound LA dysfunction, we think that this population may also stand to benefit from this therapeutic concept, as observed in this case, though further prospective study is needed.DisclosuresNone.FootnotesCirc Heart Fail is available at http://circheartfailure.ahajournals.org.Correspondence to: Barry A. Borlaug, MD, Mayo Clinic and Foundation, 200 First St SW, Rochester, MN 55905. E-mail [email protected]References1. Gibson DN, Di Biase L, Mohanty P, Patel JD, Bai R, Sanchez J, Burkhardt JD, Heywood JT, Johnson AD, Rubenson DS, Horton R, Gallinghouse GJ, Beheiry S, Curtis GP, Cohen DN, Lee MY, Smith MR, Gopinath D, Lewis WR, Natale A. Stiff left atrial syndrome after catheter ablation for atrial fibrillation: clinical characterization, prevalence, and predictors.Heart Rhythm. 2011; 8:1364–1371. doi: 10.1016/j.hrthm.2011.02.026.CrossrefMedlineGoogle Scholar2. Yang Y, Liu Q, Wu Z, Li X, Xiao Y, Tu T, Zhou S. Stiff left atrial syndrome: a complication undergoing radiofrequency catheter ablation for atrial fibrillation.J Cardiovasc Electrophysiol. 2016; 27:884–889. doi: 10.1111/jce.12966.CrossrefMedlineGoogle Scholar3. Melenovsky V, Hwang SJ, Redfield MM, Zakeri R, Lin G, Borlaug BA. 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Reddy Y, El Sabbagh A, Packer D and Nishimura R (2018) Evaluation of shortness of breath after atrial fibrillation ablation—Is there a stiff left atrium?, Heart Rhythm, 10.1016/j.hrthm.2018.01.029, 15:6, (930-935), Online publication date: 1-Jun-2018. Maeder M, Nägele R, Rohner P and Weilenmann D (2017) Pulmonary hypertension in stiff left atrial syndrome: pathogenesis and treatment in one, ESC Heart Failure, 10.1002/ehf2.12234, 5:1, (189-192), Online publication date: 1-Feb-2018. July 2017Vol 10, Issue 7 Advertisement Article InformationMetrics © 2017 American Heart Association, Inc.https://doi.org/10.1161/CIRCHEARTFAILURE.117.004160PMID: 28694356 Manuscript receivedApril 25, 2017Manuscript acceptedJune 15, 2017Originally publishedJuly 10, 2017 Keywordsleft atriumatrial fibrillationpulmonary hypertensionatrial septal defectleft atrial pressurePDF download Advertisement SubjectsCatheter-Based Coronary and Valvular InterventionsHeart FailureHemodynamicsPathophysiologyTreatment