Atrial Shunt Research Articles

Long term safety and outcomes after atrial shunting for heart failure with preserved or mildly reduced ejection fraction: 5-year and 3-year follow-up in the REDUCE LAP-HF I and II trials

BackgroundThere is a little evidence regarding long-term safety and efficacy for atrial shunt devices in heart failure (HF). MethodsThe REDUCE LAP-HF I (n=44) and II (n=621) trials (RCT-I and -II) were multicenter, randomized, sham-controlled trials of patients with HF and ejection fraction >40%. Outcome data were analyzed from RCT-I, a mechanistic trial with 5-year follow-up, and RCT-II, a pivotal trial identifying a responder group (n=313) defined by exercise PVR <1.74 WU and no cardiac rhythm management device with 3-year follow-up. ResultsAt 5 years in RCT I, there were no differences in cardiovascular (CV) mortality, HF events, embolic stroke, or new-onset atrial fibrillation between groups. After 3 years in RCT II, there was no difference in the primary outcome (hierarchical composite of CV mortality, stroke, HF events, and KCCQ) between shunt and sham in the overall trial. Compared to sham, those with responder characteristics in RCT-II had a better outcome with shunt (win ratio 1.6 [95% CI 1.2-2.2], P=0.006; 44% reduction in HF events [shunt 9 vs. control 16 per 100 patient-years], P=0.005; and greater improvement in KCCQ overall summary score [+17.9±20.0 vs. +7.6±20.4], P<0.001), while non-responders had significantly more HF events. Shunt treatment at 3 years was associated with a higher rate of ischemic stroke (3.2% vs. 0%, 95% CI 2% - 6.1%, p=0.032) and lower incidence of worsening kidney dysfunction (10.7% vs. 19.3%, p=0.041). ConclusionsWith up to 5 years of follow up, adverse events were low in patients receiving atrial shunts. In the responder group, atrial shunt treatment was associated with a significantly lower HF event rate and improved KCCQ compared to sham through 3 years of follow-up.Clinicaltrials.gov registration: NCT02600234, NCT03088033

Exploring Atrial Shunt Therapy for Heart Failure: A Comprehensive Review of the Atrial Coronary Sinus Shunt.

Heart failure is a prevalent and severe medical condition characterized by the heart's inability to pump blood efficiently, leading to poor circulation and symptoms such as pulmonary congestion. Despite advancements in medical treatments, many patients continue to experience significant symptoms with reduced quality of life. This article explores the left atrial coronary sinus shunt as an innovative interventional strategy to address hemodynamic issues in heart failure. The shunt aims to decrease left atrial pressure and alleviate pulmonary congestion by creating a connection between the left atrium and the coronary sinus.

Prophylactic perigraft to right atrial shunt controls bleeding from the right coronary ostial anastomotic leak.

Prophylactic perigraft to right atrial shunt controls bleeding from the right coronary ostial anastomotic leak.

Cyanotic heart disease due to a hypoplastic right ventricle with pulmonary valve atresia, an intact ventricular septum, with atrial level shunting in a day‐old Arabian foal

AbstractAn 8‐h‐old Arabian foal was presented for evaluation of progressive weakness since birth. The foal presented laterally recumbent with pale and muddy mucous membranes, a grade III/VI left basilar murmur, and an irregularly, irregular rhythm. Transthoracic echocardiogram along with an electrocardiogram (EKG) was performed. Atrial fibrillation was recorded throughout the examination. The left and right atria were both significantly enlarged. A right to left shunt across the atrial septum was confirmed via an intravenous injection of agitated saline. The tricuspid valve was rudimentary without apparent atrioventricular flow and no ventricular septal defect was appreciated. The animal was euthanased, and necropsy found dysplasia of the tricuspid valve, a hypoplastic right ventricle with pulmonary valve atresia, and an intact ventricular septum. This condition is similar, both haemodynamically and in terms of prognosis, to tricuspid atresia, which has been described previously in Arabian foals, with the exception that there is the complete absence of connection between the atrium and ventricle in the case of tricuspid atresia. Of 14 previously reported cases of tricuspid atresia in neonatal foals, nine were Arabian foals. Further investigation into the possibility of hypoplastic right ventricle as a familial condition in the Arabian breed should be considered.

2-Year Outcomes of an Atrial Shunt Device in HFpEF/HFmrEF: Results From REDUCE LAP-HF II

BackgroundThe REDUCE LAP-HF II (Reduce Elevated Left Atrial Pressure in Patients With Heart Failure II) trial found that, compared with a sham procedure, the Corvia Atrial Shunt did not improve outcomes in heart failure with preserved or mildly reduced ejection fraction. However, after 12-month follow-up, “responders” (peak-exercise pulmonary vascular resistance <1.74 WU and absence of a cardiac rhythm management device) were identified. ObjectivesThis study sought to determine: 1) the overall efficacy and safety of the atrial shunt vs sham control after 2 years of follow-up; and 2) whether the benefits of atrial shunting are sustained in responders during longer-term follow-up or are offset by adverse effects of the shunt. MethodsThe study analyzed 2-year outcomes in the overall REDUCE LAP-HF II trial, as well as in responder and nonresponder subgroups. The primary endpoint was a hierarchical composite of cardiovascular death or nonfatal ischemic/embolic stroke, total heart failure events, and change in health status. ResultsIn 621 randomized patients, there was no difference between the shunt (n = 309) and sham (n = 312) groups in the primary endpoint (win ratio: 1.01 [95% CI: 0.82-1.24]) or its individual components at 2 years. Shunt patency at 24 months was 98% in shunt-treated patients. Cardiovascular mortality and nonfatal ischemic stroke were not different between the groups; however, major adverse cardiac events were more common in those patients assigned to the shunt compared with sham (6.9% vs 2.7%; P = 0.018). More patients randomized to the shunt had an increase in right ventricular volume of ≥30% compared with the sham control (39% vs 28%, respectively; P < 0.001), but right ventricular dysfunction was uncommon and not different between the treatment groups. In responders (n = 313), the shunt was superior to sham (win ratio: 1.36 [95% CI: 1.02-1.83]; P = 0.037, with 51% fewer HF events [incidence rate ratio: 0.49 [95% CI: 0.25-0.95]; P = 0.034]). In nonresponders (n = 265), atrial shunting was inferior to sham (win ratio: 0.73 [95% CI: 0.54-0.98]). ConclusionsAt 2 years of follow-up in REDUCE LAP-HF II, there was no difference in efficacy between the atrial shunt and sham groups in the overall trial group. The potential clinical benefit identified in the responder group after 1 and 2 years of follow-up is currently being evaluated in the RESPONDER-HF (Re-Evaluation of the Corvia Atrial Shunt Device in a Precision Medicine Trial to Determine Efficacy in Mildly Reduced or Preserved Ejection Fraction Heart Failure) trial. (Reduce Elevated Left Atrial Pressure in Patients With Heart Failure II [REDUCE LAP-HF II]; NCT03088033)

Nonpharmacological Approaches to Managing Heart Failure With Preserved Ejection Fraction.

Heart failure with preserved ejection fraction (HFpEF) is a common subtype of heart failure marked by impaired left ventricular diastolic function and decreased myocardial compliance. Given the limited availability of evidence-based pharmacological treatments for HFpEF, there is a growing interest in nonpharmacological interventions as viable therapeutic alternatives. This review aims to explore the pathophysiology of HFpEF and present recent advancements in nonpharmacological management approaches, encompassing noninvasive therapies, invasive procedures and targeted treatments for comorbidities. An extensive literature review was undertaken to identify and synthesize emerging nonpharmacological treatment options for HFpEF, assessing their potential to enhance patient outcomes. Nonpharmacological strategies, such as vagus nerve stimulation, percutaneous pulmonary artery denervation, renal denervation, transcatheter insertion of atrial shunts and pericardial resection, demonstrate promising potential for alleviating HFpEF symptoms and improving patient prognosis. Moreover, addressing comorbidities, such as hypertension and diabetes, may offer additional therapeutic benefits. These cutting-edge techniques, in conjunction with well-established exercise therapies, pave the way for future research and clinical applications in the field. Nonpharmacological interventions hold promise for advancing HFpEF patient care and fostering a deeper understanding of these treatment approaches, which will facilitate new clinical applications and contribute to the development of more targeted therapies.

Complete Abnormal Venous Return Associated with Complex Heart Disease: Case Report S. Faiz, M. Boussif, B. Zouita, D. Basraoui, H. Jalal Published: April 27, 2024 | 4 2 DOI: 10.36347/sjmcr.2024.v12i04.035 Pages: 531-533 Downloads Abstract Total anomalous pulmonary venous return (TAPVR) is a cardiac malformation defined by a complete absence of communication between the pulmonary veins and the left atrium. All

Total anomalous pulmonary venous return (TAPVR) is a cardiac malformation defined by a complete absence of communication between the pulmonary veins and the left atrium. All oxygenated pulmonary venous return flows directly or indirectly into the right atrium. Survival is only possible via a right-to-left atrial shunt. Non-blocked forms present as high-flow shunts with moderate cyanosis. Symptoms in blocked forms are dominated by obstruction of venous return, and life-threatening distress with intense cyanosis and severe pulmonary hypertension sets in from the very first days of life. Treatment consists in connecting the pulmonary venous receptacle to the left atrium, tying off the collector and closing the inter-atrial septum. The risks of surgery remain high in severe forms, and failures usually occur in the first year of life. In the case of good surgical correction, the long-term prognosis is excellent.

Atrial Shunt Device Effects on Cardiac Structure and Function in Heart Failure With Preserved Ejection Fraction

Although the results of A Study to Evaluate the Corvia Medical Inc IASD System II to Reduce Elevated Left Atrial Pressure in Patients with Heart Failure (REDUCE LAP-HF II) trial were neutral overall, atrial shunt therapy demonstrated potential efficacy in responders (no latent pulmonary vascular disease and no cardiac rhythm management device). Post hoc analyses were conducted to evaluate the effect of shunt vs sham stratified by responder status. To evaluate the effect of atrial shunt vs sham control on cardiac structure/function in the overall study and stratified by responder status. This was a sham-controlled randomized clinical trial of an atrial shunt device in heart failure with preserved ejection fraction (HFpEF)/HF with mildly reduced EF (HFmrEF). Trial participants with evaluable echocardiography scans were recruited from 89 international medical centers. Data were analyzed from April 2023 to January 2024. Atrial shunt device or sham control. Changes in echocardiographic measures from baseline to 1, 6, 12, and 24 months after index procedure. The modified intention-to-treat analysis of the REDUCE LAP-HF II trial included 621 randomized patients (median [IQR] age, 72.0 [66.0-77.0] years; 382 female [61.5%]; shunt arm, 309 [49.8%]; sham control arm, 312 [50.2%]). Through 24 months, 212 of 217 patients (98%) in the shunt arm with evaluable echocardiograms had patent shunts. In the overall trial population, the shunt reduced left ventricular (LV) end-diastolic volume (mean difference, -5.65 mL; P <.001), left atrial (LA) minimal volume (mean difference, -2.8 mL; P =.01), and improved LV systolic tissue Doppler velocity (mean difference, 0.69 cm/s; P <.001) and LA emptying fraction (mean difference, 1.88 percentage units; P =.02) compared with sham. Shunt treatment also increased right ventricular (RV; mean difference, 9.58 mL; P <.001) and right atrial (RA; mean difference, 9.71 mL; P <.001) volumes but had no effect on RV systolic function, pulmonary artery pressure, or RA pressure compared with sham. In the shunt arm, responders had smaller increases in RV end-diastolic volume (mean difference, 5.71 mL vs 15.18 mL; interaction P =.01), RV end-systolic volume (mean difference, 1.58 mL vs 7.89 mL; interaction P =.002), and RV/LV ratio (mean difference, 0.07 vs 0.20; interaction P <.001) and larger increases in transmitral A wave velocity (mean difference, 5.08 cm/s vs -1.97 cm/s; interaction P =.02) compared with nonresponders randomized to the shunt, suggesting greater ability to accommodate shunted blood through the pulmonary circulation enabling LA unloading. In this post hoc analysis of the REDUCE LAP-HF II trial, over 2 years of follow-up, atrial shunting led to reverse remodeling of left-sided chambers and increases in volume of right-sided chambers consistent with the shunt flow but no change in RV systolic function compared with sham. Changes in cardiac structure/function were more favorable in responders compared with nonresponders treated with the shunt, supporting the previously identified responder group hypothesis and mechanism, although further evaluation with longer follow-up is needed. ClinicalTrials.gov Identifier: NCT03088033.

Invasive haemodynamics predict outcomes in paediatric pulmonary artery hypertension.

Invasive haemodynamics are often performed for initiating and guiding pulmonary artery hypertension therapy. Little is known about the predictive value of invasive haemodynamic indices for long-term outcomes in children with pulmonary artery hypertension. We aimed to evaluate invasive haemodynamic data to help predict outcomes in paediatric pulmonary artery hypertension. Patients with pulmonary artery hypertension who underwent cardiac catheterisation (2006-2019) at a single centre were included. Invasive haemodynamic data from the first cardiac catheterisation and clinical outcomes were reviewed. The combined adverse outcome was defined as pericardial effusion (due to right ventricle failure), creation of a shunt for pulmonary artery hypertension (atrial septal defect or reverse Pott's shunt), lung transplant, or death. Among 46 patients with a median [interquartile range (IQR)] age of 13.2 [4.1-44.7] months, 76% had CHD. Median mean pulmonary artery pressure was 37 [28-52] mmHg and indexed pulmonary vascular resistance was 6.2 [3.6-10] Woods units × m2. Median pulmonary artery pulsatility index was 4.0 [3.0-4.7] and right ventricular stroke work index was 915 [715-1734] mmHg mL/m2. After a median follow-up of 2.4 years, nine patients had a combined adverse outcome (two had a pericardial effusion, one underwent atrial level shunt, one underwent reverse Pott's shunt, and six died). Patients with an adverse outcome had higher systolic and mean pulmonary artery pressures, higher diastolic and transpulmonary pressure gradients, higher indexed pulmonary vascular resistance, higher pulmonary artery elastance, and higher right ventricular stroke work index (p < 0.05 each). Invasive haemodynamics (especially mean pulmonary artery pressure and diastolic pressure gradient) obtained at first cardiac catheterisation in children with pulmonary artery hypertension predicts outcomes.

An Unusual Presentation of Partial Atrioventricular Canal Defect in an Elderly Woman

A partial atrioventricular canal defect (PAVCD) is a form of endocardial cushion defect. It’s a rare congenital cardiac malformation, few reported patients have survived into the seventh decade of life. A 71-year-old female with a history of diabetes and hypertension was admitted for heart failure (HF) symptoms. Transthoracic echocardiography revealed a left to right shunt in the basal portion of the interatrial septum, 19mm ostium primum atrial septal defect (ASD), a mitral valve cleft, and severe mitral valve regurgitation. PAVCD consists of an ostium primum ASD and the dividing of the common atrioventricular (AV) valve into 2 separate AV orifices, resulting in a so-called cleft in the mitral valve. The ventricular septum is intact. Echocardiography is the key tool for diagnosis and anatomic classification. Surgical repair is recommended in adults with primum ASDs causing impaired functional capacity, right atrial or right ventricular enlargement, and left-to-right shunt without cyanosis, as long as systolic pulmonary artery pressures are lower than 50% of systemic pressures. Reconstructive surgery includes, in addition to the closure of the interatrial septum, a suturing of the mitral cleft. The estimated mean age at death of the patients with ostium primum ASD is about 40 years. The objective of this clinical case is to highlight the good clinical tolerance of atrial shunts and the anatomical particularities in the PAVCD.

Atrial septal defect closure in children at young age is beneficial for left ventricular function.

Atrial septal defects (ASDs) lead to volume-loaded right ventricles (RVs). ASD closure does not always alleviate symptoms or improve exercise capacity, which is possibly explained by impaired left ventricular (LV) haemodynamics. This study evaluated the effect of ASD closure in children using non-invasive LV pressure-volume (PV) loops derived from cardiac magnetic resonance (CMR) imaging and brachial blood pressure, compared with controls. Twenty-three children with ASD underwent CMR, and 17 of them were re-examined 7 (6-9) months after ASD closure. Twelve controls were included. Haemodynamic variables were derived from PV loops by time-resolved LV volumes and brachial blood pressure. After ASD closure, LV volume increased [76 (70-86) vs. 63 (57-70) mL/m2, P = 0.0001]; however, it was still smaller than in controls [76 (70-86) vs. 82 (78-89) mL/m2, P = 0.048]. Compared with controls, children with ASD had higher contractility [2.6 (2.1-3.3) vs. 1.7 (1.5-2.2) mmHg/mL, P = 0.0076] and arterial elastance [2.1 (1.4-3.1) vs. 1.4 (1.2-2.0) mmHg/mL, P = 0.034]. After ASD closure, both contractility [2.0 (1.4-2.5) mmHg/mL, P = 0.0001] and arterial elastance [1.4 (1.3-2.0) mmHg/mL, P = 0.0002] decreased. Despite the left-to-right atrial shunt that leads to low LV filling and RV enlargement, the LV remains efficient and there is no evidence of impaired LV haemodynamics in children. Closure of ASD at young age while the ventricle is compliant is thus beneficial for LV function. LV volumes, however, remain small after ASD closure, which may impact long-term cardiovascular risk and exercise performance.

Transcatheter Closure of Postoperative Residual Atrial or Ventricular Septal Shunts in Patients with Congenital Heart Disease

Transcatheter Closure of Postoperative Residual Atrial or Ventricular Septal Shunts in Patients with Congenital Heart Disease

Long-Term Effects Of Atrial Shunt Device On Cardiac Structure And Function In Heart Failure With Preserved And Mildly Reduced Ejection Fraction: Results From The REDUCE LAP-HF II Randomized Clinical Trial

Long-Term Effects Of Atrial Shunt Device On Cardiac Structure And Function In Heart Failure With Preserved And Mildly Reduced Ejection Fraction: Results From The REDUCE LAP-HF II Randomized Clinical Trial

Surgical right pulmonary artery to left atrial shunt for severe pulmonary hypertension and bridge to lung transplantation

Surgical right pulmonary artery to left atrial shunt for severe pulmonary hypertension and bridge to lung transplantation

Obesity in heart failure with preserved ejection fraction: Insights from the REDUCE LAP-HF II trial.

Obesity is causally related to the development of heart failure with preserved ejection fraction (HFpEF) but complicates the diagnosis and treatment of this disorder. We aimed to determine the relationship between severity of obesity and clinical, echocardiographic and haemodynamic parameters in a large cohort of patients with documented HFpEF. The REDUCE LAP-HF II trial randomized 626 patients with ejection fraction ≥40% and exercise pulmonary capillary wedge pressure (PCWP) ≥25 mmHg to atrial shunt or sham procedure. We tested for associations between body mass index (BMI), clinical characteristics, cardiac structural and functional abnormalities, physical limitations, quality of life and outcomes with atrial shunt therapy. Overall, 60.9% of patients had BMI ≥30 kg/m2 . As the severity of obesity increased, symptoms (Kansas City Cardiomyopathy Questionnaire score) and 6-min walk distance worsened. More severe obesity was associated with lower natriuretic peptide levels despite more cardiac remodelling, higher cardiac filling pressures, and higher cardiac output. Lower cut points for E/e' were needed to identify elevated PCWP in more obese patients. Strain measurements in all four chambers were maintained as BMI increased. Pulmonary vascular resistance at rest and exercise decreased with higher BMI. Obesity was associated with more first and recurrent heart failure events. However, there was no significant interaction between obesity and treatment effects of the atrial shunt. Increasing severity of obesity was associated with greater cardiac remodelling, higher right and left ventricular filling pressures, higher cardiac output and increased subsequent heart failure events. Despite significant obesity, many HFpEF patients have preserved right heart and pulmonary vascular function and thus, may be appropriate candidates for atrial shunt therapy.

Percutaneous Strategies in Structural Heart Diseases: Focus on Chronic Heart Failure.

Central Illustration : Percutaneous Strategies in Structural Heart Diseases: Focus on Chronic Heart Failure Transcatheter devices for monitoring and treating advanced chronic heart failure patients. PA: pulmonary artery; LA: left atrium; AFR: atrial flow regulator; TASS: Transcatheter Atrial Shunt System; VNS: vagus nerve stimulation; BAT: baroreceptor activation therapy; RDN: renal sympathetic denervation; F: approval by the American regulatory agency (FDA); E: approval by the European regulatory agency (CE Mark). Innovations in devices during the last decade contributed to enhanced diagnosis and treatment of patients with cardiac insufficiency. These tools progressively adapted to minimally invasive strategies with rapid, widespread use. The present article focuses on actual and future directions of device-related diagnosis and treatment of chronic heart failure.

Short-term study of atrial shunt and improvement of functional mitral regurgitation

BackgroundThis study used an atrial septal shunt to compare the treatment progress and prognosis for patients with heart failure (HF) who have different ejection fractions.MethodsTwenty HF patients with pulmonary hypertension, who required atrial septal shunt therapy, were included in this study. The patients underwent surgery between December 2012 and December 2020. They were divided into two groups based on their ejection fraction: a group with reduced ejection fraction (HFrEF) and a group with preserved ejection fraction(HFpEF) + mid-range ejection fraction (HfmrEF). Echocardiography was utilized to evaluate parameters such as left ventricular dimension (LVD), left ventricular ejection fraction (LVEF), and left ventricular end-diastolic volume (LVEDV). Hemodynamic parameters were measured using cardiac catheterization. The patient's cardiac function was assessed using the six-minute walking test (6MWT), KCCQ score, NYHA classification, and the degree of functional mitral regurgitation (FMR). Followed-up visits were conducted at 1, 3, and 6 months, and any adverse effects were recorded.ResultsThe LVEF values were consistently higher in the HFpEF+HFmrEF group than HFrEF group at all periods (P < 0.05). Differences in LVD were observed between the two groups before the surgery. Statistically, significant differences were found at the preoperative stage, 1 month, and 3 months (P < 0.05, respectively). However, the LVEDV showed a significant difference between the two groups only at 3 months (P = 0.049). Notably, there were notable variations in LAPm, LAPs, and the pressure gradient between the LA-RA gradient at baeline, after implantation, and during the 6 months follow-up (all P < 0.05).ConclusionFollowing treatment, the HFpEF+HFmrEF group exhibited more significant improvements in echocardiographic and cardiac catheterization indices than the HFrEF group. However, there was no statistically significant difference between the two groups regarding the 6MWT and KCCQ scores. It is important to note that the findings of this study still require further investigation in a large sample size of patients.

Postoperative Left Atrial Shunt in a Child with Ventricular Septal Defect Repair

Postoperative Left Atrial Shunt in a Child with Ventricular Septal Defect Repair

New method for quantification of atrial septum defect shunting by echocardiography

Abstract Background Atrial septum defect (ASD) is a common congenital heart disease. Echocardiography is the preferred method for detection and characterization of ASD. Accurate quantification of shunting is pivotal, but often requires right heart catheterization (RHC). In the present study, we introduce a new non-invasive method for accurate quantification of the ASD shunt by combined transesophageal- (TEE) and transthoracic (TTE) echocardiography. Shunt by RHC was used as a reference method. Method In 22 adults with secundum ASD, left ventricular (LV) stroke volume (SV) was measured by the pulsed Doppler method using 2D TTE. The shunt volume passing through the ASD was quantified as the ASD area measured by planimetry from 3D TEE, multiplied with the time velocity integral (TVI) of the PW Doppler obtained at the center of the ASD by TEE. Finally, the ratio of the pulmonary to systemic shunt (Qp:Qs) was calculated as (LVSV+ASDvolume)/LVSV. Shunt by RHC was assessed by the Fick’s principle. Results Mean shunt was 2.2±0.7 (±SD) by echocardiography and 2.1±0.7 by RHC, with a mean difference of -0.1±0.4 (Figure 1). The correlation between shunt measured by RHC and echocardiography was strong (r=0.86, p&amp;lt;0.0001; Figure 2). Conclusion The present study demonstrates that ASD shunting can be quantified accurately by combining TTE and TEE, suggesting a new non-invasive approach in settings where RHC is not feasible or available.Figures

Abstract 15843: Biatrial and Recurrent Cardiac Myxoma in a Rare Case of Carney Complex

Primary cardiac tumors (CTs) are exceptionally rare, and are predominantly benign masses. Myxomas make up more than 60% of these lesions and most frequently develop in the left atrium (LA), followed by the right atrium (RA). Carney complex (CNC) is a rare, typically autosomal dominant, multiple endocrine neoplasia characterized by cutaneous findings, myxomas, endocrine and/or nonendocrine neoplasms. We report a rare case of CNC involving biatrial and recurrent myxomas. A 36-year-old female presented to Cardiology Clinic at our center for management of CNC. Three years prior, she was evaluated at a local hospital for acute stroke where TTE showed a myxoma (2 x 2 x 2.5cm) attached to the left atrial septum, prompting resection. On followup TTE 2 years later, she had a mass in the LA, prolapsing through the mitral valve into the left ventricle. She underwent resection of the myxoma (2.9 x 3.9 cm) and left atrial appendage, which was complicated by recurrent stroke. She was found to have a PRKAR1A gene mutation (c.97 deletion) suggestive of CNC and was referred to our center. At Cardiology Clinic, she denied chest pain, dyspnea, presyncope, and syncope. Her father and children did not have the gene mutation. Exam revealed lentigines on her lower lip and pigmentation of an inner canthus. She was diagnosed with primary pigmented nodular adrenal disease by Endocrinology. On TEE, she was found to have a recurrent LA mass (1 x 1 x 0.8 cm) attached to the interatrial septum, but no right-to-left atrial shunt. This prompted surgery consultation. Numerous intracardiac myxomas were removed during surgery; one in the LA attached to the septum, 3-4 smaller ones, and one 2 cm myxoma located in the RA. The interatrial septum was also resected given recurrent myxoma in this region. She underwent cryoablation to prevent recurrence and did well post-surgery. CTs, which includes primary CTs and secondary metastatic disease, are uncommon where the former has an incidence of 0.001-0.3%. We presented a case of recurrent and biatrial cardiac myxomas in the setting of CNC, which is seldomly reported in the literature. Taken together, given the rarity of primary CTs, genetic predispositions or syndromes should be considered especially in the setting of recurrence and/or multiple masses.