Systemic Atrioventricular Valve Research Articles

Cardiopulmonary Bypass through a Left Thoracotomy Using Venous Drainage from the Innominate Vein in Congenital Heart Surgery

Cardiopulmonary bypass (CPB) through a left lateral thoracotomy is a useful approach for some congenital heart procedures, although vascular access for the arterial and venous cannulation can be challenging in the selective patients. Six patients underwent successful extracorporeal circulation through a left lateral thoracotomy using the innominate vein for venous drainage. No operative deaths or major complications occurred. Venous drainage solely from the innominate vein was adequate to establish partial bypass without the need for pericardiotomy. Total bypass was established with combined venous drainage from the innominate vein and the main pulmonary artery. Exposure of the systemic atrioventricular valve was excellent through a left thoracotomy. Venous drainage from the innominate vein without using atrial drainage can safely be used for extracorporeal circulation through a left lateral thoracotomy without compromising the procedure and it is a useful approach to congenital heart surgery in selected patients.

Older children at time of norwood operation have ongoing mortality vulnerability that continues after the cavopulmonary connection

Background Delayed 1st stage palliation of children with hypoplastic left heart syndrome (HLHS) and related pathologies can be associated with poor outcomes due to development of progressive pulmonary vascular disease) and volume load effects on systemic ventricle and atrioventricular valve (AVV). We examine current era survival of this patients’ subgroup. Methods Fifty-five infants >2 weeks old underwent Norwood surgery (2003-07). Separate competing risks analyses were performed to model outcomes (death, transition to next stage) after Norwood and after bidirectional cavo-pulmonary connection (BCPC). Results Median age was 32 days (range:15-118). 47% had HLHS, 53% had other complex univentricular variants. Mean ascending aorta was 4.4 ± 1.9 mm, 10% had impaired ventricle function, 11% moderate AVV regurgitation, 32% restrictive pulmonary venous return. Pulmonary blood flow was established via aortopulmonary shunt (30) or Sano shunt (25) Following Norwood, patients required longer ventilation, more oxygen & nitric oxide and had higher inotropic scores compared to traditional management protocol. Competing risks analysis showed that 2-years after Norwood 39% had died and 57% underwent BCPC. 4-years after BCPC, 15% had died and 85% underwent Fontan operation. Overall 3 years survival post-Norwood was 53%. Factors associated with mortality were age, lower weight at Norwood, impaired ventricular function, longer circulatory arrest and lower pre-BCPC saturation. Conclusions Children >2 weeks old undergoing Norwood operation frequently require postoperative pulmonary vasodilatation and high inotropic support. A significant hazard of death persists through all steps of multi-stage palliation. Elevated pulmonary vascular resistance, volume load effects such as systemic ventricle impairment and AVV regurgitation are commonly evident in patients who fail or don’t qualify to proceed to next stage palliation. Those patients should be closely monitored for timely referral for heart transplantation when indicated.

The natural and unnatural history of the systemic right ventricle in adult survivors

The study objective was to evaluate long-term trends in morbidity and mortality in a national cohort of adult patients with a systemic right ventricle due to the atrial switch for transposition of the great arteries or congenitally corrected transposition of the great arteries. We performed a retrospective cohort study from a baseline of 18 years, including life table and Kaplan-Meier analysis for probability of death/transplant, arrhythmia, surgical or percutaneous intervention, and permanent pacemaker insertion. A total of 97 adults with transposition of the great arteries-atrial switch (Mustard procedure in 80/Senning procedure in 17) and 32 adults with congenitally corrected transposition of the great arteries survived. The median ages at latest follow-up were 29 and 34 years, respectively. At 40 years of follow-up, freedom from death or transplant was 0.90 for those with transposition of the great arteries-atrial switch and 0.84 for those with congenitally corrected transposition of the great arteries (P=.833). Freedom from arrhythmia at 40 years of follow-up was 0.51 for those with transposition of the great arteries-atrial switch and 0.93 for those with congenitally corrected transposition of the great arteries (P=.007). Freedom from intervention at 40 years of follow-up was 0.33 for those with transposition of the great arteries-atrial switch after initial repair and 0.53 for those with congenitally corrected transposition of the great arteries (P=.938). Freedom from pacemaker insertion at 40 years of follow-up was 0.77 for those with transposition of the great arteries-atrial switch and 0.62 for those with congenitally corrected transposition of the great arteries (P=.161). Those patients who survive to adulthood with a systemic right ventricle experience low mortality and good functional status up to 40 years of age. However, there is a substantial burden of atrial tachyarrhythmia, and this occurs significantly earlier in those with transposition of the great arteries-atrial switch. Management of atrial tachyarrhythmia, along with systemic right ventricular dysfunction and systemic atrioventricular valve regurgitation, is likely to be the major challenge for this group of patients over the next decade.

Cardiac resynchronization therapy in a patient with congenitally corrected transposition of the great arteries and 2:1 atrioventricular block

Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart disease with an atrioventricular and ventriculo-arterial discordance in which the morphological right ventricle supports the systemic circulation and the morphological tricuspid valve is the systemic atrioventricular valve. Heart rhythm disturbances and ventricular dysfunction related to electromechanical dyssynchrony are common in adult congenital heart disease patients with a systemic right ventricle. Thus, these patients may require conventional pacemaker implantation, which in the presence of ventricular dysfunction and conduction disease may further compromise cardiac performance. Indeed, cardiac resynchronization therapy may be an effective treatment option for these patients. We report the case of a patient with CCTGA and moderate depression of systemic ventricular systolic function who developed a 2:1 atrioventricular block and was treated with cardiac resynchronization therapy.

Long-term Outcome Following Pregnancy in Women with a Systemic Right Ventricle: Is the Deterioration due to Pregnancy or a Consequence of Time?

The right ventricle (RV) supports the systemic circulation in patients who have had an intraatrial repair of transposition of the great arteries or have congenitally corrected transposition. There is concern about the ability of a systemic RV to support the additional volume load of pregnancy, and previous studies have reported deterioration in RV function following pregnancy. However, conditions with a systemic RV are also associated with progressive RV dysfunction over time. To date, no study has examined whether the deterioration associated with pregnancy is due to the physiological changes of pregnancy itself, or is part of the known deterioration that occurs with time in these patients. Women who had undergone pregnancy under the care of the Adult Congenital Heart Disease Unit at the Queen Elizabeth Hospital were retrospectively identified and matched to separate male and nulliparous female controls. Functional status (New York Health Association [NYHA]), RV function, and systemic atrioventricular valve regurgitation were recorded for each group at baseline, postpregnancy (or at 1 year for control groups) and at latest follow-up. Eighteen women had 31 pregnancies (range 1-4) resulting in 32 live births. There were no maternal but one neonatal death. At baseline, there was no significant difference in NYHA class or RV function between pregnancy and control groups. In postpregnancy, there was a significant deterioration in the pregnant group alone for both NYHA class (P = 0.004) and RV function (P = 0.02). At latest follow-up, there was a significant deterioration in RV function in all three groups. There was still a reduction from baseline in NYHA of women who had undergone pregnancy (P = 0.014), which again was not seen in the controls groups. This study suggests that pregnancy is associated with a premature deterioration in RV function in women with a systemic RV. These women are also more symptomatic, with a greater reduction in functional class compared with patients with a systemic RV who do not undergo pregnancy. This study will allow this cohort of women to be more accurately counseled as to the potential long-term risks of pregnancy.

Three-dimensional echocardiographic evaluation of quadricuspid systemic atrioventricular valve

A 56-year-old man became progressively dyspnoeic on exertion. He underwent an echocardiogram, which identified congenitally corrected transposition of the great arteries. Two-dimensional echocardiography showed that the systemic right ventricle was severely dilated with severely reduced systolic function. The left atrium was severely dilated (120 mL/m2). The systemic atrioventricular valve (tricuspid) …

Use of the Damus-Kaye-Stansel procedure prevents increased ventricular strain in Fontan candidates

In Fontan candidates, we have recently been aggressively performing the Damus-Kaye-Stansel procedure (DKS) to prevent increased afterload on the systemic ventricle. The present study investigated the efficacy of the DKS procedure in terms of the ventricular function following a Fontan operation. Patients undergoing a Fontan operation were divided into three groups: DKS performed at the time of the bidirectional Glenn or Fontan operation (DKS group, n = 25); DKS not performed at any stage due to mild pulmonary stenosis (PS) (PS group, n = 23) and DKS not performed due to pulmonary atresia (PA) or severe PS (PA group, n = 24). Ventricular function, afterload on the systemic ventricle and atrioventricular valve regurgitation were compared between groups. Cardiac catheterization before a Glenn or Fontan operation and at 1-year following the Fontan revealed significantly decreased ventricular end-diastolic pressure in the DKS group. The ventricular ejection fraction was significantly deteriorated in the PS group. Effective arterial elastance, as an index of total ventricular afterload, was increased after a Fontan in all groups, with a substantial increase in the PS group. As an index of ventricular mechanical efficiency, ventriculoarterial coupling was significantly increased only in the PS group. Cardiac ultrasonography revealed atrioventricular valve regurgitation above grade 3 persisting in many patients from the PS group. Semilunar valve function after DKS operation did not progress to moderate or worse in any patients. The proactive performance of the DKS procedure prevents increased ventricular afterload, avoiding deterioration of cardiac function and contributing to improved long-term results following a Fontan operation.

Risk factors for postoperative arrhythmia in patients with physiologic univentricular hearts undergoing Fontan procedure

Advanced age, dilated right atrium, increased preoperative pulmonary artery pressure, increased right atrial pressure, technique of operation, and poor ventricular function were reported to be risk factors for postoperative arrhythmia. Aim of this study is to determine the risk factor for postoperative arrhythmia after Fontan operation with regard to ventricle dominancy and hemodynamic parameters. In this retrospective study, the data of the patients including age, weight, dominant ventricle, type of cardiac anomaly, previous operations, duration of cardiopulmonary bypass (CPB), duration of aortic clamping, cardiac rhythm, pulmonary artery pressure, the Nakata Index, systemic atrioventricular (AV) valve insufficiency were obtained from the hospital records, the echocardiographic files, and cardiac catheterization records. Patients were assigned to dominant left ventricle or dominant right ventricle groups. Statistical analysis was performed using the Mann-Whitney U test. Arrhythmia was observed in 21 (52.5%) patients in the postoperative period. No postoperative arrhythmia was observed in patients with a right atrial pressure of <5 mmHg, whereas postoperative arrhythmias were observed in patients with a right atrial pressure of ≥5 mmHg (p<0.05). When the effects of preoperative and postoperative pulmonary artery pressures on postoperative arrhythmia were evaluated, postoperative arrhythmia was determined in only 2 (12.5%) of 8 patients with a preoperative mean pulmonary artery pressure of ≤9 mmHg, whereas postoperative arrhythmia was observed in 19 (59.3%) of 32 patients with a preoperative pulmonary artery pressure of ≥10 mmHg. A preoperative mean pulmonary artery pressure of >10 mmHg increased the risk of postoperative arrhythmia (p<0.02). Postoperative arrhythmia was determined in 8 (53%) of 15 patients with a dominant right ventricle, and in 13 (52%) of 25 patients with a dominant left ventricle (p>0.05). While a preoperative mean pulmonary artery pressure of >10 mmHg and a preoperative right atrial pressure of >5 mmHg were the risk factors for postoperative arrhythmia in patients undergoing Fontan procedure, the right or left ventricular dominance was not a risk factor.

Catheter Ablation of Atrial Fibrillation in Transposition of the Great Arteries Treated With Mustard Atrial Baffle

The survival of patients with D-transposition of the great arteries (D-TGA) has dramatically improved with corrective surgical procedures, including the atrial and arterial switch operations. In the current era, the majority of the adult patients typically have undergone atrial switch operations, either a Senning or a Mustard procedure. Late complications include baffle obstruction, systemic ventricular failure, systemic atrioventricular valve regurgitation, and rhythm disturbances. The most common atrial arrhythmia in this population is intra-atrial reentrant tachycardia (IART), which has been associated with development of heart failure and death.1–4 Proarrhythmic factors include atrial enlargement from tricuspid regurgitation and systemic right ventricular failure, as well as suture lines. As this population ages, chronic hemodynamic stress may result in atrial fibrillation as well. We present the first report of catheter ablation for treatment of atrial fibrillation in a patient with D-TGA treated with prior Mustard repair. ### Case Report A 41-year-old man with D-TGA status post Mustard repair presented with longstanding, recurrent atrial arrhythmias. He originally underwent balloon atrial septostomy 2 days after birth and a subsequent atrial switch procedure using the Mustard technique at 2 years of age.5 He did well until age 32, at which time he had recurrent symptomatic IART. EP study was performed. Cavotricuspid isthmus (CTI)-dependent flutter was induced and confirmed by entrainment mapping. Lines of ablation were drawn across the systemic venous baffle portion of the “medial” CTI and the pulmonary venous “lateral” CTI using a retrograde, aortic approach. Flutter terminated with ablation and could not be reinduced. Bidirectional block was demonstrated across the CTI. He remained arrhythmia-free for 2 years, then presented again at age 34 with exertional intolerance and fatigue due to partial obstruction of the pulmonary venous baffle. He underwent patch augmentation of the stenotic …

SYSTEMIC RIGHT VENTRICULAR DYSFUNCTION IN CONGENITALLY CORRECTED TRANSPOSITION OF THE GREAT ARTERIES: IS THE ATRIOVENTRICULAR VALVE ALWAYS THE CULPRIT?

Systemic right ventricular (SRV) dysfunction and systemic atrioventricular valve (SAVV) regurgitation are common in adult patients (pt) with congenitally corrected transposition of the great arteries (CCTGA). Our aim was to determine the prevalence and relationship between SAVV regurgitation and SRV

Late diagnosis of corrected transposition of the great arteries in an elderly patient with coronary artery disease

Congenitally corrected transposition of the great arteries (c-TGA) is a rare congenital heart disease usually diagnosed during childhood. Rarely, some patients remain undiagnosed and develop symptoms of heart failure during adulthood due to dysfunction of the left ventricle and systemic atrioventricular valve. We report on a 69-year-old male patient with a history of percutaneous coronary intervention and stent implantation for coronary artery disease and coronary artery anomaly, in whom diagnosis of c-TGA was missed.

Congenitally Corrected Transposition of the Great Arteries: Ventricular Function at the Time of Systemic Atrioventricular Valve Replacement Predicts Long-Term Ventricular Function

Congenitally Corrected Transposition of the Great Arteries: Ventricular Function at the Time of Systemic Atrioventricular Valve Replacement Predicts Long-Term Ventricular Function

In vitro comparison of different mechanical prostheses suitable for replacement of the systemic atrioventricular valve in children

The aim of the present study was to compare the hydrodynamics of 4 different mechanical prostheses fitting the atrioventricular annulus in children. We tested different inverted aortic prostheses with a prosthesis-annulus relationship in the mitral chamber of the Sheffield pulse duplicator (Department of Medical Physics and Clinical Engineering, Royal Hallamshire Hospital, Sheffield, UK), analyzed by comparing the prosthetic housing diameter and the predicted annulus diameter based on body surface area (0.8 and 1 m(2) corresponding to an annulus diameter of 18.8-20.2 mm). The On-X 19 (On-X Life Technologies, Inc, Austin, Tex), SJM Regent 19 (St Jude Medical Inc, St Paul, Minn), Sorin Overline 18 (Sorin Biomedica, Saluggia, Italy), and Medtronic Advantage Supra 19 (Medtronic Inc, Minneapolis, Minn) valves with a housing diameter of 19 to 20 mm were hydrodynamically compared. The tests were carried out at increasing pulse rate of 72, 80, 100, and 120 beats/min for a stroke volume of 20 and 30 mL. Therefore, cardiac output ranged from 1.44 to 3.6 L/min. Regardless of the pulse rate and stroke volume, the Medtronic Advantage Supra valve showed the highest mean diastolic pressure difference at each cardiac output (P<.05). The mean gradients were significantly lower for the Sorin Overline valve regardless of the cardiac output, stroke volume, and pulse rate (P<.05). The effective orifice areas observed followed exactly the same behavior: the lowest for the Medtronic Advantage Supra valve and the highest for the Sorin Overline valve. The Sorin Overline valve showed the highest closure volumes (P<.05), and the On-X prosthesis showed the highest leakage volumes (P<.05). The Sorin Overline valve had the highest total regurgitant volume (P<.05), and the Medtronic Advantage Supra valve had the lowest total regurgitant volume (P<.05). The On-X valve showed the highest total energy loss regardless of the pulse rate at 20 mL of stroke volume, which was comparable to the SJM Regent and Sorin Overline valves at increased stroke volume. The Medtronic Advantage Supra valve showed the lowest total energy loss regardless of cardiac outputs (P<.05). This hydrodynamic evaluation model allowed us to compare the efficiency of currently available valve prostheses suitable for atrioventricular replacement in children. Among these prostheses, the Sorin Overline valve showed the best diastolic performance. On the other hand, for total energy loss, the Medtronic Advantage Supra valve demonstrated excellent performance.

Impact of Intraoperative Epicardial Real-Time Three-Dimensional Echocardiography as a Surgical Guide on Systemic Atrioventricular Valve Plasty of Congenital Heart Disease

The aim of the study is to assess the feasibility and accuracy of intraoperative epicardial RT3DE (IERT3DE) for obtaining anatomical information of systemic atrioventricular valve as a surgical guide in congenital heart disease.

Congenitally Corrected Transposition of the Great Arteries: Ventricular Function at the Time of Systemic Atrioventricular Valve Replacement Predicts Long-Term Ventricular Function

The objective was to evaluate the systemic ventricular ejection fraction (SVEF) at the time of systemic atrioventricular valve (SAVV) replacement as a predictor of SVEF ≥1 year after surgery in patients with congenitally corrected transposition of the great arteries (CCTGA). Progressive SAVV regurgitation causes systemic ventricular failure in CCTGA patients, who are commonly referred late for intervention. Survival after surgery is poor when the pre-operative SVEF is <44%. We retrospectively reviewed 46 patients (pre-operative SVEF ≥ 40% in 27 patients and <40% in 19 patients) with 2 good-sized ventricles, a morphologically right systemic ventricle, and SAVV regurgitation requiring surgery. Median follow-up was not different in patients with a pre-operative SVEF ≥ 40% (8.8 years) or <40% (7.7 years, p = 0.36). Pre-operative SVEF was the only independent predictor of ≥ 1-year post-operative SVEF (p < 0.0001). The late SVEF was preserved (defined as ≥ 40%) in 63% of patients who underwent surgery with an SVEF ≥ 40% compared with 10.5% of patients who underwent surgery with an SVEF <40%. Pre-operative variables associated with late mortality were an SVEF ≤ 40%, a subpulmonary ventricular systolic pressure ≥ 50 mm Hg, atrial fibrillation, and New York Heart Association functional class III to IV. Post-operative systemic ventricular function after SAVV replacement can be predicted from the pre-operative SVEF. For best results, operation should be considered at an earlier stage, before the SVEF falls below 40% and the subpulmonary ventricular systolic pressure rises above 50 mm Hg.

An Unusual Case of Kartagener Syndrome

A 45-year-old man had been diagnosed of Kartagener syndrome (KS) in infancy, involving situs inversus or ‘‘mirror’’ position of the abdominal organs (cardiac magnetic resonance images: Figs. 1A, B, C and D. L, liver; G, gastric fundus) with levocardia (Fig. 1E, arrow), associated with congenitally corrected transposition of the great arteries (CTGA), and right aortic arch (Figs. 1C and F, arrow). The anatomic pattern of CTGA was visceroatrial situs inversus (I), ventricular D-loop with atrioventricular and ventriculoarterial discordance (D), and Dtransposition of the great arteries (D) (Fig. 1F). Owing to severe failure of the systemic atrioventricular valve and dysfunction of the systemic ventricle, the patient required valve replacement surgery at age 38 years. A metal prosthesis was implanted without complications (Figs. 1E, 2A and B, arrowhead. SV, systemic ventricle; PV, pulmonary ventricle; LA, left atrium; RA, right atrium). Since that time, he has undergone periodic follow-up and has remained asymptomatic. Kartagener syndrome is a recessive autosomal genetic condition with an incidence of 1 case in 32 000 to 68 000 live births. It is characterized by the triad of situs inversus (often with dextrocardia), chronic sinusitis, and bronchiectasis. An uncommon anomaly that accounts for less than 1% of all congenital heart diseases, CTGA occurs in a typical criss-cross pattern (S, L, L). However, in the rare cases associated with KS showing situs inversus and dextrocardia, the expected pattern is I, L, L. The patient described is the first reported case of KS with levocardia associated with CTGA (I, D, D), right aortic arch, and valve replacement. A B C

Older children at the time of the Norwood operation have ongoing mortality vulnerability that continues after cavopulmonary connection

Delayed first-stage palliation of children with hypoplastic left heart syndrome and related pathologies can be associated with poor outcomes because of development of progressive pulmonary vascular disease and volume load effects on the systemic ventricle and atrioventricular valve. We examine the current era's survival in this subgroup. Fifty-five infants older than 2 weeks underwent the Norwood operation (2003-2007). Separate competing risk analyses were performed to model outcomes (death and transition to the next stage) after the Norwood operation and after bidirectional cavopulmonary connection. Median age was 32 days (range, 15-118 days). Forty-seven percent had hypoplastic left heart syndrome, and 53% had other complex univentricular variants. Mean ascending aortic size was 4.4 ± 1.9 mm, 10% had impaired ventricular function, 11% had moderate atrioventricular valve regurgitation, and 32% had restrictive pulmonary venous return. Pulmonary blood flow was established through an aortopulmonary shunt (n = 30) or Sano shunt (n = 25). After the Norwood operation, patients required longer ventilation and more oxygen and nitric oxide and had higher inotropic scores compared with those undergoing the traditional management protocol. Competing risks analysis showed that 2 years after the Norwood operation, 39% had died, and 57% underwent bidirectional cavopulmonary connection. Four years after bidirectional cavopulmonary connection, 15% had died, and 85% underwent the Fontan operation. Overall 3-year survival after the Norwood operation was 53%. Factors associated with mortality were age, lower weight at the time of the Norwood operation, impaired ventricular function, longer circulatory arrest, and lower pre-bidirectional cavopulmonary connection saturation. Children older than 2 weeks undergoing the Norwood operation frequently require postoperative pulmonary vasodilatation and high inotropic support. A significant hazard of death persists through all steps of multistage palliation. Increased pulmonary vascular resistance and volume load effects, such as systemic ventricular impairment and atrioventricular valve regurgitation, are commonly evident in patients in whom treatment fails or who do not qualify to proceed to the next stage of palliation. Those patients should be closely monitored for timely referral for heart transplantation when indicated.

Significance of the residual aortic obstruction in multistage repair of hypoplastic left heart syndrome

The population of children who received right ventricle-to-pulmonary artery shunt (RV-PA) at Norwood procedure (NP) is growing, but limited data are available regarding the long-term outcome. The aim of this study was to present operative outcomes and mid-term hemodynamics and to assess the impact of the residual aortic obstruction on the results in patients undergoing three-staged surgery with RV-PA application at NP. Between June 2001 and June 2009, 229 children with hypoplastic left heart syndrome (HLHS) and variants underwent NP with RV-PA; 172 have proceeded to stage II and 95 to stage III. The medical records (clinical data, echocardiographic records, cardiac catheterization reports, electrocardiograms, and surgical notes) were retrospectively reviewed. The later era of NP was associated with significantly better outcome (2001-2004: 1- and 5-years' survival were 64.3% and 59.8%, respectively; 2005-2009: 1- and 4-years' survival were 93.1% and 86.9%, respectively) (p<0.001). There was no association between long-term survival and diagnosis (HLHS/HLHS variant) (p=0.39). The incidence of depressed ventricular function and moderate or severe systemic atrioventricular valve regurgitation among the children who required balloon aortoplasty (BA) before stage II surgery was significantly higher than in children without aortic arch obstruction (p=0.027, p=0.008, respectively). In midterm follow-up, BA had no significant influence on the actuarial survival (p=0.089). No ventricular arrhythmias were noticed at any stage. The RV-PA shunt is a safe technique that does not seem to impair systolic or electrical ventricular function; its outcomes continue to improve with growing experience. Combined cardiologic interventional and surgical procedures are required to optimize the outcomes.

Situs inversus with levocardia and congenitally corrected transposition of great vessels with rheumatic tricuspid valve stenosis and regurgitation

Congenitally corrected transposition of great arteries is a rare congenital anomaly. This case report describes a 30-year-old patient of congenitally corrected transposition of the great arteries with rheumatic involvement of systemic (tricuspid) atrio-ventricular valve.

Congenitally Corrected Transposition of the Great Arteries and Concomitant Coronary Artery and Valvular Disease in the Adult Patient

Congenitally corrected transposition of the great arteries (ccTGA) accounts for less that 1% of cardiac anomalies, and is defined as ventriculoarterial and atrioventricular (AV) discordance. The double discordant connection allows for survival with the right ventricle performing as the systemic ventricle, and the left ventricle as the pulmonary ventricle. We report a case of ccTGA in a 35-year-old male with situs inversus totalis status post repair of a ventricular septal defect (VSD) with a residual VSD, severe systemic AV valve regurgitation, and coronary artery disease who presented with chest pain. He subsequently underwent tricuspid valve replacement and VSD repair, followed by percutaneous coronary revascularization. This case highlights many important issues of adults with congenital cardiac disease, as well as the specific surgical management of anomalies associated with ccTGA. We review the literature and discuss the management of these complicated patients.