How Artificial Intelligence Can Enhance the Diagnosis of Cardiac Amyloidosis: A Review of Recent Advances and Challenges.

Cardiac amyloidosis (CA) is an underdiagnosed form of infiltrative cardiomyopathy caused by abnormal amyloid fibrils deposited extracellularly in the myocardium and cardiac structures. There can be high variability in its clinical manifestations, and diagnosing CA requires expertise and often thorough evaluation; as such, the diagnosis of CA can be challenging and is often delayed. The application of artificial intelligence (AI) to different diagnostic modalities is rapidly expanding and transforming cardiovascular medicine. Advanced AI methods such as deep-learning convolutional neural networks (CNNs) may enhance the diagnostic process for CA by identifying patients at higher risk and potentially expediting the diagnosis of CA. In this review, we summarize the current state of AI applications to different diagnostic modalities used for the evaluation of CA, including their diagnostic and prognostic potential, and current challenges and limitations.

Non-invasive detection of cardiac allograft rejection among heart transplant recipients using an electrocardiogram based deep learning model.

Aims: Current non-invasive screening methods for cardiac allograft rejection have shown limited discrimination and are yet to be broadly integrated into heart transplant care. Given electrocardiogram (ECG) changes have been reported with severe cardiac allograft rejection, this study aimed to develop a deep-learning model, a form of artificial intelligence, to detect allograft rejection using the 12-lead ECG (AI-ECG). Methods and results: Heart transplant recipients were identified across three Mayo Clinic sites between 1998 and 2021. Twelve-lead digital ECG data and endomyocardial biopsy results were extracted from medical records. Allograft rejection was defined as moderate or severe acute cellular rejection (ACR) based on International Society for Heart and Lung Transplantation guidelines. The extracted data (7590 unique ECG-biopsy pairs, belonging to 1427 patients) was partitioned into training (80%), validation (10%), and test sets (10%) such that each patient was included in only one partition. Model performance metrics were based on the test set (n = 140 patients; 758 ECG-biopsy pairs). The AI-ECG detected ACR with an area under the receiver operating curve (AUC) of 0.84 [95% confidence interval (CI): 0.78-0.90] and 95% (19/20; 95% CI: 75-100%) sensitivity. A prospective proof-of-concept screening study (n = 56; 97 ECG-biopsy pairs) showed the AI-ECG detected ACR with AUC = 0.78 (95% CI: 0.61-0.96) and 100% (2/2; 95% CI: 16-100%) sensitivity. Conclusion: An AI-ECG model is effective for detection of moderate-to-severe ACR in heart transplant recipients. Our findings could improve transplant care by providing a rapid, non-invasive, and potentially remote screening option for cardiac allograft function.

Sirolimus-Based Immunosuppression Is Associated with Decreased Incidence of Post-Transplant Lymphoproliferative Disorder after Heart Transplantation: A Double-Center Study.

Mammalian target of rapamycin (mTOR) inhibitors have been shown to reduce proliferation of lymphoid cells; thus, their use for immunosuppression after heart transplantation (HT) may reduce post-transplant lymphoproliferative disorder (PTLD) risk. This study sought to investigate whether the sirolimus (SRL)-based immunosuppression regimen is associated with a decreased risk of PTLD compared with the calcineurin inhibitor (CNI)-based regimen in HT recipients. We retrospectively analyzed 590 patients who received HTs at two large institutions between 1 June 1988 and 31 December 2014. Cox proportional-hazard modeling was used to examine the association between type of primary immunosuppression and PTLD after adjustment for potential confounders, including Epstein-Barr virus (EBV) status, type of induction therapy, and rejection. Conversion from CNI to SRL as primary immunosuppression occurred in 249 patients (42.2%). During a median follow-up of 6.3 years, 30 patients developed PTLD (5.1%). In a univariate analysis, EBV mismatch was strongly associated with increased risk of PTLD (HR 10.0, 95% CI: 3.8-26.6; p < 0.001), and conversion to SRL was found to be protective against development of PTLD (HR 0.19, 95% CI: 0.04-0.80; p = 0.02). In a multivariable model and after adjusting for EBV mismatch, conversion to SRL remained protective against risk of PTLD compared with continued CNI use (HR 0.12, 95% CI: 0.03-0.55; p = 0.006). In conclusion, SRL-based immunosuppression is associated with lower incidence of PTLD after HT. These findings provide evidence of a benefit from conversion to SRL as maintenance therapy for mitigating the risk of PTLD, particularly among patients at high PTLD risk.

Cardiac Transplantation and Consecutive Minimally Invasive Pectus Excavatum Repair.

Pectus excavatum is a common chest wall deformity with inward deviation of sternum and accompanying ribs. The depression can cause symptomatic cardiac compression, although the cardiopulmonary impact remains controversial. We present 2 cases of cardiac transplantation followed by modified minimally invasive pectus excavatum repair due to the hemodynamic consequences of the pectus deformity.

Edge-to-edge tricuspid valve repair for severe tricuspid regurgitation 20 years after cardiac transplantation.

Tricuspid valve regurgitation in orthotopic heart transplant recipients is common. Surgical corrections have been the mainstay of the treatment for diuretic-refractory heart failure due to severe tricuspid regurgitation. However, post-transplant patients inherently carry higher surgical risk owing to previous sternotomy and immunocompromised state. We report a case of successful percutaneous edge-to-edge tricuspid valve repair for severe tricuspid regurgitation after cardiac transplantation. A 27-year-old man with a history of idiopathic restrictive cardiomyopathy status after orthotopic heart transplant presented with severe right-sided heart failure symptoms. A transthoracic echocardiogram showed bi-atrial enlargement and moderate-to-severe tricuspid regurgitation, and an increase to the severe range with exercise. Percutaneous edge-to-edge tricuspid valve repair was performed. The patient's symptoms improved, and follow-up echocardiogram showed mild tricuspid regurgitation. Percutaneous tricuspid valve repair can be considered as an alternative option to conventional surgery for symptomatic severe tricuspid regurgitation in orthotopic heart transplant recipients with suitable anatomy.

Successful Use of Intragastric Balloon Therapy as a Bridge to Heart Transplantation.

Obesity is an important limiting factor for heart transplantation (HT) in patients with congestive heart failure (CHF). Achieving substantial weight loss is challenging in this population due to activity limitations and fluid retention. Endoscopic bariatric therapies (EBTs) including intragastric balloons (IGB) are effective primary weight loss therapies. IGBs have also been successfully utilized as a bridge therapy prior to liver transplantation and, in one case report, prior to HT. Potential advantages of IGBs in this population include low bleeding risk and less invasiveness as compared to other EBTs and surgery. We report the successful use of IGB as a bridge therapy in two patients with class II obesity and end-stage CHF requiring left ventricular assist devices (LVAD), anticoagulation, antiplatelet, and inotrope therapy.

Early Postoperative Aortic Root Thrombus After Heartmate 3.

As a bridge to heart transplantation or destination treatment, implantation of the Heartmate 3 (HM3) left ventricular assist device is a viable option for patients with end-stage congestive heart failure. The recent Momentum 3 trial has shown favorable outcomes compared with Heartmate 2. We report the first case of aortic root thrombus occurring early after HM3 implantation as a bridge to heart transplantation. Our case suggests that bridging with an Impella 5.0 preceding HM3 implantation could potentially predispose patients to aortic root thrombus after HM3 implantation, due to Impella-related injury to the aortic valve and aortic root stasis after durable LVAD support.

Factors associated with adverse outcomes from cardiovascular events in the kidney transplant population: an analysis of national discharge data, hospital characteristics, and process measures.

BACKGROUND: Kidney transplant (KT) patients presenting with cardiovascular (CVD) events are being managed increasingly in non-transplant facilities. We aimed to identify drivers of mortality and costs, including transplant hospital status. METHODS: Data from the 2009-2011 Nationwide Inpatient Sample, the American Hospital Association, and Hospital Compare were used to evaluate post-KT patients hospitalized for MI, CHF, stroke, cardiac arrest, dysrhythmia, and malignant hypertension. We used generalized estimating equations to identify clinical, structural, and process factors associated with risk-adjusted mortality and high cost hospitalization (HCH). RESULTS: Data on 7803 admissions were abstracted from 275 hospitals. Transplant hospitals had lower crude mortality (3.0% vs. 3.8%, p = 0.06), and higher un-adjusted total episodic costs (Median $33,271 vs. $28,022, p < 0.0001). After risk-adjusting for clinical, structural, and process factors, mortality predictors included: age, CVD burden, CV destination hospital, diagnostic cardiac catheterization without intervention (all, p < 0.001). Female sex, race, documented co-morbidities, and hospital teaching status were protective (all, p < 0.05). Transplant and non-transplant hospitals had similar risk-adjusted mortality. HCH was associated with: age, CVD burden, CV procedures, and staffing patterns. Hospitalizations at transplant facilities had 37% lower risk-adjusted odds of HCH. Cardiovascular process measures were not associated with adverse outcomes. CONCLUSION: KT patients presenting with CVD events had similar risk-adjusted mortality at transplant and non-transplant hospitals, but high cost care was less likely in transplant hospitals. Transplant hospitals may provide better value in cardiovascular care for transplant patients. These data have significant implications for patients, transplant and non-transplant providers, and payers.

Destination Therapy: Standardizing the Role of Palliative Medicine and Delineating the DT-LVAD Journey.

CONTEXT: Destination therapy (DT) patients face significant challenges as they transition from chronic left ventricular assist device (LVAD) support to comfort-oriented care. Integration of palliative medicine (PM) into the multidisciplinary team is important to facilitate advanced care planning (ACP) and improve quality of life (QoL). OBJECTIVES: We evaluated the impact of a structured programmatic approach to the end-of-life (EOL) process in DT patients as measured by QoL surveys and the utilization of ACP. METHODS: We instituted a four prong intervention approach: 1) delineated the path from implant to EOL by defining specific stages, including a transitional phase where care limits were agreed upon, 2) standardized the role of PM, 3) held transitional care meetings to support shared decision-making, and 4) held multidisciplinary team debriefings to facilitate communication. Preintervention and postintervention outcomes were measured for patients/caregivers by using the QUAL-E/QUAL-E (family) QoL instrument. Wilcoxon signed-ranks test compared nonparametric variables. RESULTS: All patients (n = 41)/caregivers (n = 28) reported improved QoL measures (patient P = 0.035/caregiver P = 0.046). Preparedness plans increased from 52% to 73% after implementation and advance directives increased from 71% to 83%. Fifty-nine percent of the patients completed an outpatient PM clinic visit; 51% completed/scheduled a second visit. Clinician outcomes improved including satisfaction with multidisciplinary team communication/expectations, ACP processes, and EOL management. CONCLUSION: A programmatic approach that standardizes the role of PM and delineates the patient's path from implant to EOL improved quality outcomes and increased implementation of ACP. A defined communication process allowed the multidisciplinary team to have a clear patient management approach.

Myocardial Contraction Fraction by M-Mode Echocardiography Is Superior to Ejection Fraction in Predicting Mortality in Transthyretin Amyloidosis.

BACKGROUND: Transthyretin amyloidosis (ATTR) is often associated with cardiac involvement manifesting as conduction disease as well as restrictive cardiomyopathy causing heart failure and death. Myocardial contraction fraction (MCF), the ratio of left ventricular stroke volume (SV) to myocardial volume (MV), is a volumetric measure of myocardial shortening that is superior to ejection fraction (EF) in predicting mortality in patients with primary amyloid light chain cardiac amyloidosis. We hypothesized that MCF would be an independent predictor of survival in TTR-CA. METHODS AND RESULTS: MCF was derived from 2-dimensional echocardiography-guided M-mode data for 530 subjects in the Transthyretin Amyloidosis Outcomes Survey (THAOS) database: age 61 ± 16years, 74% male, 158 wild-type (ATTRwt) and 372 mutant (ATTRm), follow-up 1.5 ± 1.7years. Using multivariate Cox proportional hazard regression models, MCF <25% was highly associated with survival (hazard ratio [HR] 8.5, 95% confidence interval [CI] 4.8-14.9,-P < .0001), which was stronger than the association of EF dichotomized at 50% (HR 2.8, 95% CI 1.8-4.4; P < .0001). MCF <25% remained significantly predictive of survival in a multivariate model that included systolic blood pressure, estimated glomerular filtration rate <65 mL·min-1·m-2, New York Heart Association (NYHA) functional class, and health status based on the EuroQol-5D-3L questionnaire (area under the receiver operating characteristic curve [AUC] = 0.83, 95% CI 0.78-0.89). CONCLUSIONS: MCF was superior to EF in predicting mortality in patients with ATTR. A predictive model combining MCF with systolic blood pressure, renal function, NYHA functional class, and health status was strongly associated with survival in patients with ATTR. CLINICALTRIALS. GOV IDENTIFIER: NCT00628745.

Cardiorespiratory Fitness (Peak Oxygen Uptake): Safe and Effective Measure for Cardiovascular Screening Before Kidney Transplant.

BACKGROUND: Significant heterogeneity exists in practice patterns and algorithms used for cardiac screening before kidney transplant. Cardiorespiratory fitness, as measured by peak oxygen uptake (VO2peak), is an established validated predictor of future cardiovascular morbidity and mortality in both healthy and diseased populations. The literature supports its use among asymptomatic patients in abrogating the need for further cardiac testing. METHODS AND RESULTS: We outlined a pre-renal transplant screening algorithm to incorporate VO2peak testing among a population of asymptomatic high-risk patients (with diabetes mellitus and/or >50 years of age). Only those with VO2peak <17 mL/kg per minute (equivalent to <5 metabolic equivalents) underwent further noninvasive cardiac screening tests. We conducted a retrospective study of the a priori dichotomization of the VO2peak <17 versus ≥17 mL/kg per minute to determine negative and positive predictive value of future cardiac events and all-cause mortality. We report a high (>90%) negative predictive value, indicating that VO2peak ≥17 mL/kg per minute is effective to rule out future cardiac events and all-cause mortality. However, lower VO2peak had low positive predictive value and should not be used as a reliable metric to predict future cardiac events and/or mortality. In addition, a simple mathematical calculation documented a cost savings of ≈$272 600 in the cardiac screening among our study cohort of 637 patients undergoing evaluation for kidney and/or pancreas transplant. CONCLUSIONS: We conclude that incorporating an objective measure of cardiorespiratory fitness with VO2peak is safe and allows for a cost savings in the cardiovascular screening protocol among higher-risk phenotype (with diabetes mellitus and >50 years of age) being evaluated for kidney transplant.

Soluble ST2 does not change cardiovascular risk prediction compared to cardiac troponin T in kidney transplant candidates.

BACKGROUND: Solubility of Tumorigenicity 2 (sST2) is a novel biomarker that better stratifies risk of cardiovascular events (CVE) compared to cardiac troponin T(cTnT) in heart failure. We assessed the association of sST2 with the composite outcome of CVE and/or mortality compared to cTnT in kidney transplant candidates. METHODS: 200 kidney transplant candidates between 2010 and 2013 were included. Elevated sST2 was defined as ≥30ng/ml, cTnT≥0.01 ng/ml. RESULTS: Median age 53 (interquartile range (IQR) 42-61) years, 59.7% male and 82.0% white. 33.5% had history of CVE, 42.5% left ventricular hypertrophy (LVH) and 15.6% positive cardiac stress test. Elevated sST2 correlated with male gender, history of prior-transplants, CVE, positive stress test, LVH, elevated cTnT, anemia, hyperphosphatemia, increased CRP and non-transplanted status. Male gender, history of CVE and LVH were independent determinants of sST2. During 28 months (IQR 25.3-30), 7.5% died, 13.0% developed CVE and 19.0% developed the composite outcome. Elevated sST2 was associated with the composite outcome (hazard ratio = 1.76, CI 1.06-2.73, p = 0.029) on univariate analysis but not after adjusting for age, diabetes and cTnT (p = 0.068). sST2 did not change the risk prediction model for composite outcome after including age, diabetes, prior history of CVE and elevated cTnT. CONCLUSIONS: Increased sST2 level is significantly associated with variables associated with CVE in kidney transplant candidates. sST2 was associated with increased risk of the composite outcome of CVE and/or death but not independent of cTnT. Larger studies are needed to confirm these findings and determine whether sST2 has added value in CV risk stratification in this cohort of patients.

Patterns of Care and Outcomes in Cardiovascular Disease After Kidney Transplantation in the United States.

BACKGROUND: Cardiovascular disease (CVD) is an important driver of mortality after kidney transplantation. Its broader impact on posttransplant health care utilization in US hospitals is unknown. METHODS: We used administrative claims data from the Nationwide Inpatient Sample and the American Hospital Association Annual Survey to identify hospitalizations for kidney transplant patients with a cardiovascular diagnosis from 2005 to 2011. CVD hospitalizations were stratified by transplant hospital status to characterize patterns in inpatient health care utilization and outcomes. Based on these analyses, the domestic burden of treatment for posttransplant CVD (myocardial infarction, stroke, congestive heart failure, dysrhythmia, cardiac arrest, malignant hypertension) was estimated. RESULTS: The total domestic burden of post-kidney transplant hospitalization between 2005 and 2011 is estimated at 389 138 of which 26.5% of episodes were related to CVD (n = 103 118). CVD was responsible for a growing proportion of post-transplant hospitalizations over time (24.4%-30.4%, P < 0.001). Compared with nontransplant hospitals, transplant hospitals had similar length of stay (median length of stay, 3.7 days), higher median costs per hospitalization (US $10 364 vs US $8606, overall US $9324), and lower adjusted mortality (3.2% vs 3.9%, overall 3.6%; P = 0.003). CONCLUSIONS: Inpatient CVD care is increasing over time for kidney transplant patients, accounting for 30% of all post-transplant hospitalizations. Variation exists in the inpatient care, outcomes, and costs between by hospital type. Further studies are needed to better understand the mechanisms behind these phenomena.

Genotype and Phenotype of Transthyretin Cardiac Amyloidosis: THAOS (Transthyretin Amyloid Outcome Survey).

BACKGROUND: Transthyretin amyloidosis (ATTR) is a heterogeneous disorder with multiorgan involvement and a genetic or nongenetic basis. OBJECTIVES: The goal of this study was to describe ATTR in the United States by using data from the THAOS (Transthyretin Amyloidosis Outcomes Survey) registry. METHODS: Demographic, clinical, and genetic features of patients enrolled in the THAOS registry in the United States (n = 390) were compared with data from patients from other regions of the world (ROW) (n = 2,140). The focus was on the phenotypic expression and survival in the majority of U.S. subjects with valine-to-isoleucine substitution at position 122 (Val122Ile) (n = 91) and wild-type ATTR (n = 189). RESULTS: U.S. subjects are older (70 vs. 46 years), more often male (85.4% vs. 50.6%), and more often of African descent (25.4% vs. 0.5%) than the ROW. A significantly higher percentage of U.S. patients with ATTR amyloid seen at cardiology sites had wild-type disease than the ROW (50.5% vs. 26.2%). In the United States, 34 different mutations (n = 201) have been reported, with the most common being Val122Ile (n = 91; 45.3%) and Thr60Ala (n = 41; 20.4%). Overall, 91 (85%) of 107 patients with Val122Ile were from the United States, where Val122Ile subjects were younger and more often female and black than patients with wild-type disease, and had similar cardiac phenotype but a greater burden of neurologic symptoms (pain, numbness, tingling, and walking disability) and worse quality of life. Advancing age and lower mean arterial pressure, but not the presence of a transthyretin mutation, were independently associated with higher mortality from a multivariate analysis of survival. CONCLUSIONS: In the THAOS registry, ATTR in the United States is overwhelmingly a disorder of older adult male subjects with a cardiac-predominant phenotype. Val122Ile is the most common transthyretin mutation, and neurologic phenotypic expression differs between wild-type disease and Val122Ile, but survival from enrollment in THAOS does not. (Transthyretin-Associated Amyloidoses Outcome Survey [THAOS]; NCT00628745).

Pathology of Chronic Chagas Cardiomyopathy in the United States: A Detailed Review of 13 Cardiectomy Cases.

OBJECTIVES: The pathologic features of chronic Chagas cardiomyopathy may not be widely appreciated in the United States. We sought to describe the gross, microscopic, immunohistochemical, and molecular pathology features useful to diagnose chronic Chagas cardiomyopathy. METHODS: The features from a case series of cardiectomy specimens of patients undergoing heart transplantation (12 patients) or mechanical circulatory support device implantation (one patient) for chronic Chagas cardiomyopathy at three institutions in the United States are reported and analyzed. RESULTS: Gross findings included enlarged and dilated ventricles (100% of cases), mural thrombi (54%), epicardial plaques (42%), and left ventricular aneurysm (36%). Microscopic evaluation revealed myocarditis (100% of cases) characterized by mononuclear cell infiltration, fibrosis (100%), nonnecrotizing granulomas (62%), and giant cells (38%). Two specimens (15%) showed rare intracellular amastigotes. Immunohistochemical assays for Trypanosoma cruzi organisms were negative in all cardiectomy specimens, whereas tissue polymerase chain reaction was positive in six (54%) of 11 cases. CONCLUSIONS: The gross and microscopic features of chronic Chagas cardiomyopathy in the United States appear similar to those reported in endemic countries. Importantly, tissue polymerase chain reaction may be useful to confirm the diagnosis.

Operative management of acquired thoracic dystrophy in adults after open pectus excavatum repair.

BACKGROUND: In young children, acquired thoracic dystrophy (ATD) is associated with extensive resection of cartilage, often during open pectus excavatum (PE) repair. Progressive dyspnea or exercise intolerance may develop in these patients secondary to cardiac compression or restrictive pulmonary function. Surgical treatment of ATD by attempting to increase the overall thoracic volume has been controversial. We describe our experience with adults presenting for surgical correction of ATD. METHODS: A retrospective medical record review was performed for all patients with ATD presenting for surgical evaluation from December 2010 through February 2013. RESULTS: Ten adult male patients were evaluated for treatment of ATD after an open Ravitch procedure for PE. Nine patients, whose mean age was 34 years (range, 21-42 years), elected to proceed with surgical treatment. The mean age of the initial repair was 3.7 years. Extensive reconstruction, chest wall expansion, and placement of stainless steel support bars and titanium plating were performed in all patients. Eight patients had minor complications, and major complications occurred in 3 patients. Respiratory failure with prolonged ventilator support occurred in 3 patients. There were no reoperations or deaths. At mean follow-up of 16 months (range, 6-31 months), all patients subjectively reported improvement in their ability to exercise and in their symptoms, including dyspnea with exertion. CONCLUSIONS: ATD may be associated with early childhood Ravitch repair. Adults may present with disabling symptoms related to cardiac compression and restrictive pulmonary function. Reconstruction with sternal elevation and expansion of the anterior chest subjectively improves symptoms.

Natural history of left ventricular mechanics in transplanted hearts: relationships with clinical variables and genetic expression profiles of allograft rejection.

OBJECTIVES: The aim of this study was to explore the temporal evolution of left ventricular (LV) mechanics in relation to clinical variables and genetic expression profiles implicated in cardiac allograft function. BACKGROUND: Considerable uncertainty exists regarding the range and determinants of variability in LV systolic performance in transplanted hearts (TXH). METHODS: Fifty-one patients (mean age 53 ± 12 years; 37 men) underwent serial assessment of echocardiograms, cardiac catheterization, gene expression profiles, and endomyocardial biopsy data within 2 weeks and at 3, 6, 12, and 24 months after transplantation. Two-dimensional speckle-tracking data were compared between patients with TXH and 37 controls (including 12 post-coronary artery bypass patients). Post-transplantation mortality and hospitalizations were recorded with a median follow-up period of 944 days. RESULTS: Global longitudinal strain (LS) and radial strain remained attenuated in patients with TXH at all time points (p < 0.001 and p = 0.005), independent of clinical rejection episodes. Failure to improve global LS at 3 months (≥ 1 SD) was associated with higher incidence of death and cardiac events (hazard ratio: 5.92; 95% confidence interval: 1.96 to 17.91; p = 0.049). Multivariate analysis revealed gene expression score as the only independent predictor of global LS (R(2) = 0.53, p = 0.005), with SEMA7A gene expression having the highest correlation with global LS (r = -0.84, p < 0.001). CONCLUSIONS: Speckle tracking-derived LV strains are helpful in estimating the burden of LV dysfunction in patients with TXH that evolves independent of biopsy-detected cellular rejection. Failure to improve global LS at 3 months after transplantation is associated with a higher incidence of death and cardiac events. Serial changes in LV mechanics correlate with peripheral blood gene expression profiles and may affect the clinical assessment of long-term prognosis in patients with TXH.

Current management of pectus excavatum: a review and update of therapy and treatment recommendations.

Pectus excavatum (PE) is a posterior depression of the sternum and adjacent costal cartilages and is frequently seen by primary care providers. PE accounts for >90% of congenital chest wall deformities. Patients with PE are often dismissed by physicians as having an inconsequential problem; however, it can be more than a cosmetic deformity. Severe cases can cause cardiopulmonary impairment and physiologic limitations. Evidence continues to present that these physiologic impairments may worsen as the patient ages. Data reports improved cardiopulmonary function after repair and marked improvement in psychosocial function. More recent consensus by both the pediatric and thoracic surgical communities validates surgical repair of the significant PE and contradicts arguments that repair is primarily cosmetic. We performed a review of the current literature and treatment recommendations for patients with PE deformities.