Repercusiones cardíacas en pacientes con pectus excavatum: puesta al día. Segunda parte / Cardiac repercussions in patients with pectus excavatum: update. Part two / Repercussões cardíacas em pacientes com pectus excavatum: atualização. Parte dois

El pectus excavatum (PEX) es una deformación de la pared torácica que obedece a una alteración de los cartílagos costales con el consiguiente hundimiento del esternón. Históricamente, se clasificaba como un defecto únicamente estético o cosmético, sin embargo, en los últimos años se han desarrollado nuevos métodos de estudio para la valoración de las repercusiones de esta patología. Existe cada vez más bibliografía que demuestra importantes repercusiones funcionales. Se realizó una puesta al día de las repercusiones cardíacas de la patología y un análisis de los artículos más relevantes de los últimos años. La evidencia actual permite afirmar que existe una afectación cardíaca por compresión esternal en la mayoría de los pacientes con PEX. Las afectaciones incluyen alteraciones anatomofuncionales (trastornos del ritmo, disminución del llenado ventricular), del volumen sistólico, aumento de la presión de la aurícula derecha, valvulopatías, compresión del ventrículo derecho, derrame pericárdico, entre otras. Todo lo cual permite concluir que el PEX puede presentar importantes alteraciones cardíacas que deben ser tenidas en cuenta a la hora de valorar los pacientes con esta patología.

Pectus excavatum (PEX) is a deformation of the chest wall caused by an alteration of the costal cartilages with the consequent collapse of the sternum. Historically, it had been classified as a solely aesthetic or cosmetic defect, however, in recent years new study methods have been developed to assess the repercussions of this pathology, with increasing bibliography showing important functional consequences. We updated the cardiac pathological repercussions and analyzed the most relevant articles of recent years. The current evidence suggests that there is cardiac involvement due to sternal compression in most patients with PEX. These affectations include anatomical functional alterations: rhythm disorders, decreased ventricular filling, decreased stroke volume, increased right atrial pressure, valve disease, right ventricular compression, pericardial effusion, among others. All of which enables us to conclude that PEX can present important cardiac alterations that must be taken into account when assessing patients with this pathology.

Pectus excavatum (PEX) é uma deformação da parede torácica decorrente de uma alteração das cartilagens costais com consequente colapso do esterno. Historicamente, foi classificado como um defeito exclusivamente estético ou cosmético, porém, nos últimos anos, novos métodos de estudo foram desenvolvidos para avaliar as repercussões dessa patologia, com crescente bibliografia mostrando importantes repercussões funcionais. Foi realizada uma atualização das repercussões cardíacas da patologia e análise dos artigos mais relevantes dos últimos anos. As evidências atuais permitem afirmar que há acometimento cardíaco por compressão esternal na maioria dos pacientes com PEX. As afecções incluem alterações anatomofuncionais: distúrbios do ritmo, diminuição do enchimento ventricular, diminuição do volume sistólico, aumento da pressão atrial direita, doença valvular, compressão do ventrículo direito, derrame pericárdico, entre outras. Tudo isso permite concluir que o PEX pode apresentar alterações cardíacas importantes que devem ser levadas em consideração na avaliação de pacientes com essa patologia.

Correlation of anthropometric index and cardiopulmonary exercise testing in children with pectus excavatum.

BACKGROUND: Cardiopulmonary exercise testing (CPET) is a method used to evaluate functional impairment of patients with various diseases. OBJECTIVE: The objective was to use CPET to estimate the usability of anthropometric index (AI) in patients with pectus excavatum (PE) as a marker of functional impairment caused by chest deformity. METHODS: The study included 32 paediatric patients (28 males) with PE. Patients underwent CPET using a breath-by-breath exhaled gas analysis method and continuous monitoring of cardiac parameters. RESULTS: In both groups, two (overall four) patients met criteria for cardiogenic limitation (low VO2 and low O2Pulse). Mean VO2/WR was below two standard deviations (2SD) in patients with less severe PE; other observed parameters were within normal limits (Z-score ± 2 SD). The AI had no observed correlation with peak ventilation, VO2peak and peak workload. CONCLUSION: The obtained CPET data do not correlate well with the severity of chest deformity expressed with AI. There were similar physical activity limitations in both examined groups of patients and they did not depend on the severity of the deformity.

Comparison of physical functions and psychosocial conditions between adolescents with pectus excavatum, pectus carinatum and healthy controls.

PURPOSE: The present study aimed to compare the physical and psychosocial conditions of adolescents with pectus excavatum (PE) and pectus carinatum (PC), who had mild-severe deformities, with those of healthy controls (HC). METHODS: This study included 180 adolescents (aged 10-18 years) with pectus deformity [PE (n = 90) and PC (n = 90)] and 90 age-matched HC. The following parameters were evaluated for each participant: clinical parameters, perceived appearance of the chest area (PAC), physical functions (grip strength, flexibility, muscle strength, endurance, physical activity), posture, psychosocial conditions, and quality of life (QOL). RESULTS: Patients with PE and PC had a lower body weight, a worse, a poorer posture, lower scores for physical functions compared to the HC group (p < 0.001, all). The PE group had lower scores (p < 0.05) for some psychosocial conditions and quality of life subscales compared to the HC and PC (except for the QOL) group (p < 0.05). The PC group had a poorer posture compared to the PE group (p < 0.05). PAC was associated with physical functions and psychosocial status (r = 0.19-0.40, p < 0.05) but pectus severity was not associated with these parameters (r = 0.02-0.12, p > 0.05). CONCLUSION: Our results indicate that all adolescents with mild, moderate, or severe pectus deformity should undergo a biopsychosocial evaluation, receive psychosocial support, and be referred for physiotherapy.

The vacuum treatment for the pectus excavatum thickened subcutaneous fat of the chest wall and is effective in preteenagers.

PURPOSE: We report effects of treatment for pectus excavatum (PE) with a Vacuum Bell (VB). MATERIALS AND METHODS: Fifteen patients (13 males and 2 females) undergoing VB therapy over 6 years, aged 6-17 years (mean: 11.1 years), were divided into two Groups: Group 1 (G1), preteenagers (< 13 years); Group 2 (G2), teenagers (≧ 13 years). We retrospectively recorded changes in depth of depression and the Haller index. RESULTS: The depth of depression reduced in 93.3% of 15 patients (mean; 8.7 mm). Minimal change occurred in the Haller index but the subcutaneous fat thickened significantly (11/15 patients). The improvement rate on elevation of the chest wall was better in G1 than G2 (G1: 54.0% vs G2: 51.3%). CONCLUSION: The maximum depth of depression improved in PE patients as a result of thickening of subcutaneous fat. VB is most effective in preteenagers with PE.

The chest wall gender divide: females have better cardiopulmonary function and exercise tolerance despite worse deformity in pectus excavatum.

PURPOSE: Pectus excavatum (PE) is a chest wall deformity of variable severity and symptomatology. Existing female-specific literature highlights breast asymmetry and cosmetic reconstruction. We sought to evaluate gender differences in cardiopulmonary function. METHODS: Cardiac MRIs, pulmonary function tests (PFTs), and cardiopulmonary exercise tests (CPETs) were reviewed in 345 patients undergoing preoperative evaluation for PE. Regression modeling was used to evaluate associations between gender and clinical endpoints of cardiopulmonary function. RESULTS: Mean age was 15.2 years, 19% were female, 98% were white. Pectus indices included median Haller Index (HI) of 4.8, mean depression index (DI) of 0.63, correction index (CI) of 33.6%, and Cardiac Compression Index (CCI) of 2.79. Cardiac assessment revealed decreased right and left ventricular ejection fraction (RVEF, LVEF) in 16% and 22% of patients, respectively. PFTs and CPETs were abnormal in ~ 30% of patients. While females had deeper PE deformities-represented by higher pectus indices-they had superior function with higher RVEF, LVEF Z-scores, FEV1, VO2 max, O2 pulse, work, and breathing reserve (p < 0.05). CONCLUSION: Despite worse PE deformity and symptomatology, females had a better cardiopulmonary function and exercise tolerance than males. Further research is needed to assess the precise mechanisms of this phenomenon and postoperative outcomes in this population.

False-positive electrocardiographic changes during exercise test in a patient with pectus excavatum.

Exercise-induced ST-segment changes simulating myocardial ischemia have been described in otherwise normal subjects during hyperventilation. We present the case of a 60-year-old man with pectus excavatum showing significant exercise-induced "pseudo-ischaemic" ST-segment changes with neither coronary artery disease nor anxiety-induced hyperventilation. We found no report of the possible causative role of a narrow antero-posterior chest diameter in inducing "pseudo-ischaemic" ST-segment changes during exercise stress test in the literature.

Quadrangular Fixation of Pectus Bars to Prevent Displacement in Nuss Procedure.

BACKGROUND: Double pectus bars are sometimes inserted to correct pectus excavatum. Method of double-bar fixation to prevent bar displacement has been rarely reported. We have used quadrangular fixation of the double pectus bars. The objective of this study was to compare results of the quadrangular fixation procedure with those of the classic separate fixation procedure. METHODS: From September 2011 to January 2016, 86 patients underwent Nuss procedure with double-bar insertion. In 44 patients, each bar was fixed separately (group A). In 42 patients, quadrangular fixation of the bars was performed with metal plates (group B). Patient demographics, Haller index (HI), bar displacement index (BDI), and reoperation rate were compared between the two groups. RESULTS: The mean patient age was 17.2 years (range: 3-40 years) in group A and 17.8 years (range: 4-30 years) in group B. There was no significant difference in preoperative or postoperative HIs between the two groups (all p >0.05). Early complication rates were 15.9% in group A and 9.5% in group B (p > 0.05). In group A, three patients underwent surgery to correct bar displacement (6.8% of reoperation rate), whereas there was no corrective surgery in group B. BDIs of the two groups were significantly different (p < 0.01). CONCLUSIONS: When quadrangular fixation was performed with upper and lower pectus bars bilaterally fixed by connecting each bar with plates, bar displacement was prevented more effectively than separate fixation, thus minimizing reoperation.

Disproportion and dysmorphism in an adult Belgian population with Turner syndrome: risk factors for chronic diseases?

BACKGROUND: Turner syndrome (TS) is characterized by dysmorphism and body disproportion. TS women are also susceptible to a range of chronic disorders including arterial hypertension (AHT), osteoporosis, sensorineural hearing loss (SNHL), type 2 diabetes mellitus (DM2) and thyroid disease. The association between dysmorphism/body disproportion and chronic disease has never been studied in TS women. The effect of growth hormone treatment on body disproportion is also unclear. Objectives: to analyze dysmorphic features and body disproportion in TS women in relation to the presence of chronic disease and to document the effect of growth hormone therapy on body disproportion. METHOD: 76 adult TS women with a regular follow up at the TS clinic UZ Ghent were invited to participate. Detailed body measurements were performed in 44 volunteering TS women. Scoring systems for overall dysmorphism, craniofacial dysmorphism, thoracic and limb abnormalities and skeletal disproportion were developed. RESULTS: TS women with a higher dysmorphism score were more at risk for AHT (p = 0.04) as well as those with a higher sitting height/standing height ratio (p < 0.05). Prevalence of AHT, osteoporosis and DM 2 was lower in TS women treated with GH during childhood (p < 0.05). CONCLUSIONS: Adult TS women with relatively short legs or with more physical dysmorphic stigmata were more at risk for AHT. GH therapy does not seem to increase the risk of chronic disease on the long term.

Improved Dynamics of Thoracic Cage and Exercise Capacity after Nuss Repair for Pectus Excavatum.

BACKGROUND: Pectus excavatum (PE) reduces the dynamics of the thoracic cage, with a negative impact on exercise capacity. We aimed to evaluate the effects of Nuss repair for PE on the dynamics of the thoracic cage and exercise capacity in adults. METHODS: This was a prospective observational study of 46 adults (mean age, 26.2 years) who underwent PE correction using the Nuss procedure between September 2016 and August 2017. Cirtometry was used to obtain measures of thoracic cage circumference at two levels (axillary level [AL] and xyphoid level [XL]), at the end points of inspiration and expiration. Circumference measures were obtained before surgery and at 1, 3, and 6 months after surgery. Exercise capacity was also evaluated using the 6-minute walk test (6MWT). The association between the 6MWT data and cirtometry measures was evaluated using Pearson's correlation. RESULTS: The circumference at maximum inspiration increased from baseline to 3 months after surgery (p < 0.01), at both the AL (84.5 ± 4.9 vs. 88.5 ± 5.1 cm) and XL (80.1 ± 4.8 vs. 83.7 ± 5.1 cm). The 6MWT also significantly improved from baseline to 3 months after surgical correction (544.7 ± 64.1 vs. 637.3 ± 59.4 m, p < 0.01), with this improvement being correlated to the increase in thoracic circumference on maximal inspiration at both the AL and XL (0.8424 and 0.7951, respectively). CONCLUSION: Improved dynamics of the thoracic cage were achieved after Nuss repair for PE in adults. This increase in thoracic circumference at maximum inspiration was associated with an improvement in exercise capacity at 3 months after surgery.

Caring for People With Untreated Pectus Excavatum: An International Online Survey.

BACKGROUND: Pectus excavatum is a chest wall deformity with no known cause and no standardized guidelines for evaluation or management. There is a pressing need to characterize the symptoms that these individuals experience and to evaluate a potential mismatch between their expected and observed experiences with health care. We hypothesized that these individuals would feel that their health-care needs are not adequately met. METHODS: A total of 331 participants with untreated pectus excavatum from 47 countries recruited from the Pectus Awareness and Support Group completed a questionnaire about living with pectus excavatum. We focused on characterizing physical and psychosocial symptom frequency and whether these problems were discussed with providers during encounters related to pectus excavatum. RESULTS: A total of 46% and 31% of participants experience daily physical and psychosocial symptoms, respectively, but providers disproportionally focus on physical symptoms. Seventy-seven percent and 61% of participants thought their providers could do more to address their physical and psychosocial symptoms, respectively. Only 8% of participants were very satisfied after their most recent health-care visit about pectus excavatum. The overwhelming majority of participants have encountered providers that lacked basic knowledge about pectus. CONCLUSIONS: The results of this questionnaire expose a major discrepancy between expectations and delivery of care for people with pectus excavatum. These individuals should be routinely screened for both physical and psychosocial symptoms by general practitioners.

Improvement in Polysomnographic Objective Sleep Quality in Adults with Pectus Excavatum After the Nuss Procedure.

BACKGROUND: Patients with pectus excavatum have a poorer subjective sleep quality and quality of life than the general population. The Nuss procedure has been shown to improve these patients' quality of life, but data regarding their postoperative sleep quality are lacking. We aimed to evaluate the objective sleep quality of adults with pectus excavatum before and after the Nuss procedure. METHODS: Twenty-eight participants completed this study. Epworth Sleepiness Scale (ESS) scores for daytime sleepiness, Pittsburgh Sleep Quality Index (PSQI) scores for subjective sleep quality, and overnight polysomnography for objective sleep quality were evaluated before and 6 months after the Nuss procedure. RESULTS: Subjective sleep quality improved after the Nuss procedure. The median PSQI score decreased from 7 [interquartile range (IQR): 5; 9] to 5 (IQR: 4; 7, p = 0.029); the median percentage of poor PSQI sleep quality decreased from 64.3 to 35.7% (p = 0.048). The median percentage of rapid eye movement sleep significantly increased after surgery [15.6% (IQR: 12.2%; 19.8%) vs. 20.4% (IQR: 14.5%; 24.9%), p = 0.016]. Sleep interruptions also improved, with the median arousal index decreasing from 9.5 (IQR: 4.8; 18.2) to 8.2 (IQR: 4.3; 12.1; p = 0.045). However, there was no significant change in ESS scores after surgery (p = 0.955). CONCLUSIONS: Pectus excavatum may be associated with poor subjective and objective sleep quality in adults, and the condition may improve after the Nuss procedure. For adults with pectus excavatum who report poor subjective sleep quality, polysomnography should be considered to assess their preoperative and postoperative sleep condition.

Does aggressive respiratory rehabilitation after primary nuss procedure improve pulmonary function?

OBJECTIVE: Here, we present the efficacy of an aggressive respiratory rehabilitation program that was initiated from early postoperative period, expecting to improve inspiratory volume at an early stage after primary Nuss procedure. METHODS: The study was performed as a nonrandomized controlled study undergoing primary Nuss procedure at our institute from 2016 to 2018. We evaluated 34 patients and assigned them to two groups based on whether or not they received a respiratory rehabilitation. Group A (between June 2016 and March 2018) includes patients with inspiratory training postoperatively, and group B (between April 2018 and July 2018) includes patients without training. At the time of admission, a physiatrist provided each patient with a training instruction. From day five postoperatively, the patients in group A started training for inspiration with an incentive spirometer (Coach 2®PORTEX) four times a day. Patients were instructed to perform the same training at home after discharge. Using this device, we measured pre- and postoperative inspired volumes in each group. Data were analyzed with Wilcoxon-Mann-Whitney tests and a paired Student t-test. A p-value below 0.05 was considered statistically significant. RESULTS: There were no statistically significant differences between the two groups concerning both operative age and the degree of the deformity calculated by Haller index. Preoperative inspiratory volume showed no significant differences. Although there were no statistical differences between two groups in the inspiratory volume at discharge, these two parameters were significantly higher in group A after three months postoperatively (A: 1404 ±â€¯466 ml vs B: 900 ±â€¯314 ml) and the values increased compared to the preoperative stage in group A. CONCLUSIONS: These data indicate the aggressive respiratory rehabilitation is an effective regimen to increase inspiratory volume at an early stage after Nuss procedure. LEVEL OF EVIDENCE: III.

Preoperative multimodality imaging of pectus excavatum: State of the art review and call for standardization.

PURPOSE: Image acquisition protocols and reports in patients with pectus excavatum (PEX) differ significantly from routine examinations, and no imaging modality can enable a comprehensive assessment of PEX severity and cardiac impact within a single examination. We therefore attempt to establish recommendations about preoperative imaging in patients with PEX. METHOD: Chest computed tomography (CT), stress echocardiography (Echo), and cardiac magnetic resonance (CMR) allow the evaluation of specific information regarding structural and functional characteristics of vital importance to assess surgical candidacy and define surgical strategies. We sought to provide a multidisciplinary state of the art document involving thoracic surgeons, radiologists, and cardiologists; to establish recommendations about the variables to be included in the reports of the imaging examinations performed in patients with PEX. RESULTS: We provide recommendations for preoperative image acquisition and analysis, aimed at the assessment of the severity of the chest wall deformity (CT); the site of maximum cardiac compression, extent of increased interventricular dependence, and presence of pericardial effusion (CMR); and the effect of PEX on the functional capacity and exercise-related systolic and/or diastolic function, and tricuspid annulus compression (Echo). CONCLUSIONS: This multidisciplinary state of the art document involving thoracic surgeons, radiologists, and cardiologists provides recommendations about preoperative imaging for patients with PEX.

Characteristics of mitral valve leaflet length in patients with pectus excavatum: A single center cross-sectional study.

The mitral valve morphology in patients with pectus excavatum (PE) has not been fully investigated. Thirty-five patients with PE, 46 normal controls, and patients with hypertrophic cardiomyopathy (HCM) who underwent 2 leaflet length measurements of Carpentier classification P2 and A2 using a transthoracic echocardiography were retrospectively investigated. The coaptation lengths and depths, papillary muscle tethering length, and mitral annular diameters were also measured. The P2 and A2 lengths were separately compared between 2 groups: older than 16 years and 16 years or younger. Furthermore, the correlations between actual P2 or A2 lengths and Haller computed tomography index, an index of chest deformity, were investigated in patients with PE exclusively. Among subjects older than 16 years, patients with PE had significantly shorter P2, longer A2, shorter copatation depth, and longer papillary muscle tethering length compared with normal controls. Similarly, patients with PE had significantly shorter P2 and shorter coaptation depth even compared with patients with HCM, while no significant difference was found in A2 length and papillary muscle tethering length. The same tendency was noted between 4 normal controls and 7 age- and sex-matched patients with PE ≤ 16 years old. No significant difference regarding A2/P2 ratio was found between patients with PE older and younger than 16 years. No significant correlation between the Haller computed tomography index and actual mitral leaflet lengths in patients with PE older than 16 years was noted; the same was observed for A2/P2 in all patients with PE. In conclusion, the characteristic features of the shorter posterior mitral leaflet, the longer anterior mitral leaflet, the shorter coaptation depth, and the longer papillary muscle tethering length in patients with PE was demonstrated. This finding might provide a clue regarding the etiology of mitral valve prolapse in PE at its possible earliest form.

Pulmonary function in children with Pectus excavatum and post-operative changes after nuss procedure.

PURPOSE: The aim of this study was to show the difference between the pulmonary function of children with mild or severe types of pectus excavatum (PE) and post-operative changes in their predicted vital capacity (%VC) following Nuss procedure. PATIENTS AND METHODS: One hundred and twenty-four of the 208 children who underwent Nuss procedure for PE between January 2007 and March 2016 were deemed eligible for this study and evaluated retrospectively. A spirometry was performed on these children on four occasions: before operation; pre-bar removal; and 1 month and 1 year after bar removal. Ninety-seven of the children who were more than 7 years of age were divided using the Haller Index (HI) into a mild group (n = 54) and a severe group (n = 43) and compared. The children were also divided into three groups based on their age and their %VC was compared at each follow-up occasion. RESULTS: The severe group showed a significantly lower %VC and peak expiratory flow rate than the mild group. %VC change after bar removal showed significantly lower in group aged 11 or over. 43 of the children had spirometry data recorded 1 year after bar removal which, compared with 1 month after bar removal, showed a significant higher %VC in groups aged of 10 or under. CONCLUSION: Nuss procedure in children aged of 10 or under proved to be an advantage in the post-operative pulmonary function.

The physiologic impact of pectus excavatum repair.

The adverse physiologic effects of pectus excavatum and subsequent resolution following correction have been a subject of controversy. There are numerous accounts of patients reporting subjective improvement in exercise tolerance after surgery, but studies showing clear and consistent objective data to corroborate this phenomenon physiologically have been elusive. This is partially due to a lack of consistent study methodologies but even more so due to a mere paucity of data. As experts in the repair of pectus excavatum, it is not uncommon for pediatric surgeons to operate on adult patients. For this reason, this review evaluates the contemporary literature to provide an understanding of the physiologic impact of repairing pectus excavatum on pediatric and adult patients separately.

Nuss procedure in the adult population for correction of pectus excavatum.

Minimally invasive repair of pectus excavatum has been successfully modified for use in adult patients. Many patients present in adulthood with progression of symptoms as the chest wall becomes less compliant with age. A thorough workup is completed including echocardiogram and chest CT to evaluate for anatomic abnormalities. Cardiopulmonary exercise testing is done to quantify the physiologic impact. Modifications of the original Nuss procedure required to allow for successful adult repair include the use of forced sternal elevation, the use of multiple bars, medial bar fixation, and interspace support to prevent bar rotation and migration. Occasionally, fractures may occur that require an open procedure and osteotomy or cartilage resection and hybrid approach incorporating the principals of intrathoracic support and osteotomy with bar stabilization.

Outcomes, quality of life, and long-term results after pectus repair from around the globe.

The procedure introduced by Donald Nuss in 1997 at the American Pediatric Surgical Association meeting has now been adopted by pediatric, thoracic, and plastic surgeons around the globe. Since its introduction, the Nuss procedure has been the subject of intense scrutiny. More than 20 years since the original publication, medical centers from around the world have reported their experience with the procedure and robust evidence now supports its safety and efficacy. Additionally, in collaboration with psychologists in Norfolk, a methodology was devised to measure the psychosocial as well as the physical benefits of the procedure. Extensive independent multinational and multi-institutional data demonstrate repair of pectus excavatum using the Nuss procedure results in marked improvement in both physical function and body image. This report reviews the outcomes, quality of life, and long-term results of numerous centers worldwide since the introduction of the procedure.

Cardiopulmonary Function in Thoracic Wall Deformities: What Do We Really Know?

Patients with pectus excavatum (PE) frequently present with complaints of exercise intolerance and cardiopulmonary symptoms. There continues to be controversy regarding the physiologic benefits of repair. The aim of this review is to summarize and discuss recent data regarding the cardiopulmonary effects of PE deformity and the evidence for improvement obtained after surgical repair including (1) a greater efficiency of breathing (chest wall mechanics), (2) improvement in pulmonary restrictive deficits, (3) an increase in cardiac chamber size and output, with improved cardiac strain and strain rate, and (4) improvement in exercise capacity.

Paroxysmal Atrial Fibrillation in Patients Successfully Treated by Radiofrequency Catheter Ablation with Severely Compression, Lateral Displacement, and Clockwise Rotation of Their Hearts due to Severe Pectus Excavatum.

Two cases with severe pectus excavatum and symptomatic atrial fibrillation (AF) underwent radiofrequency catheter ablation (RFCA). Their chest X-ray and computed tomography (CT) findings revealed lateral displacement and clockwise rotation of their hearts, and severe right atrial and mild right ventricular compression against the sternum, but no left atrium compression against the spinal column. The procedure was therefore carefully performed under guidance with CT, intra-cardiac echography, atriography, and a three-dimensions mapping system. Finally, the AF was successfully treated by RFCA without any complications. These findings underscore the importance of understanding cases of abnormal anatomy and carefully designing a strategy before performing any procedure.