Congenitally Corrected Transposition of the Great Arteries: “When the Valve Leaks, the Ventricle Speaks”

They say the heart has a mind of its own. In the rare case of congenitally corrected transposition of the great arteries (ccTGA), it quite literally has a mind of its own – and sometimes, it decides to rearrange its plumbing. This rare congenital condition, where the right and left sides of the heart swap roles – plays Musical Chairs, but keep the blood flowing in the right direction, is nature’s way of saying, “Why make it simple when you can make it interesting?” The recently accepted case report in this issue of the Journal of Clinical and Preventive Cardiology, December 2025, sheds light on a 56-year-old man who walked through half a century unaware that his heart had played musical chairs. Eleven years after a pacemaker implantation for complete heart block (CHB), the curtain lifted, revealing ccTGA, the grandmaster of anatomical deception. It was a surprise waiting in the wings. ccTGA, initially described by Von Rokitansky in 1875, accounts for <1% of all congenital heart defects, with an incidence of ∼ 1 in 33,000 births, that is rarer than finding a needle in a haystack while wearing boxing gloves and it is defining feature double discordance (atrioventricular and ventriculoarterial discordance), sounds like a cardiologist’s tongue twister, but it keeps the systemic and pulmonary circuits in series, preserving life despite the flip. Yet, over time, the morphologic right ventricle (RV), designed for the low-pressure pulmonary circuit, tires out when pushed into the high-pressure world of systemic circulation. About 13 different anatomic subtypes of ccTGA have been described according to the presence/absence of associated ventricular septal defects (VSDs), pulmonary stenosis or atresia, tricuspid valvular lesions, or atrial septal defects.[1] The patient’s story underscores an important clinical truth – even when the heart beats in rhythm, its wiring may not follow the textbook. Conduction abnormalities, particularly CHB, can be the tip of the CCTGA iceberg. About 45% of patients develop conduction issues, and 2% per year may progress to CHB. If you do not look for ccTGA, it will happily hide in plain sight. The systemic RV in ccTGA, valiant though it is, was never built for the long haul. Over decades, it begins to falter – like a marathoner forced to sprint endlessly uphill. Tricuspid regurgitation, the loyal companion of systemic RV dysfunction, accelerates the - “When the valve leaks, the ventricle speaks.” What makes this report special is not just the diagnosis but the missed opportunities. A decade earlier, when the pacemaker was implanted, a detailed Echocardiogram might have unmasked the anomaly. But hindsight, as they say, has 20/20 vision – or in this case, a perfect Echo window. Adult congenital heart disease (ACHD) has long been the neglected cousin of pediatric cardiology, but times are changing. The 2025 AHA Scientific Update on ACHD emphasizes the “need for vigilance” in adults with unexplained heart block or heart failure, recommending comprehensive imaging – echocardiogram, cardiac computed tomography, or magnetic resonance imaging – to rule out concealed congenital anomalies.[1-3] Medicine, at its heart, is about pattern recognition. Yet ccTGA breaks the mold – it teaches humility. Every murmur has a story, every pacemaker has a reason, and every “normal” heart may be a masterpiece of anatomical improvisation. Looking forward, the management of systemic RV failure in ccTGA remains a clinical conundrum. Should we replace the tricuspid valve early, before the RV gives up? Or should we wait until symptoms surface? Evidence in 2025 continues to favor early surgical intervention before the ejection fraction dips below 40%. In comedy and cardiology, timing is everything. For clinicians, this case serves as a wake-up call: think congenital even in adults. For patients, it’s a testament that the heart, despite its quirks, remains remarkably resilient. And for medical science, it’s a gentle reminder that the map of the human heart still holds a few unmapped territories. “Don’t judge a book by its cover and a heart by its chambers.” Because sometimes, the heart that beats against all odds is not just corrected by nature-it corrects our assumptions. In the end, congenital or corrected, the heart teaches us a timeless truth: resilience beats perfection. In a world obsessed with normalcy, ccTGA stands as a metaphor for life itself: beautifully imperfect, wonderfully functional, and occasionally, delightfully out of sync. In ccTGA - double discordance, circulation works for a while - but the price is paid later when the morphologic right ventricle (mRV) must shoulder the systemic load, a classic case of the “wrong man for the wrong job”. Early surgical strategies focused on physiological repair, addressing associated lesions like VSD closure, pulmonic stenosis, tricuspid valve repair while leaving the mRV as the systemic ventricle. However, by adulthood, many patients develop Progressive Systemic Right Ventricular Failure, Tricuspid Regurgitation (TR), arrhythmias, increased mortality, morbidity. Enter the anatomic repair, or the famed double switch operation—two wrongs finally making a right. By combining an atrial switch (Senning/Mustard) with an arterial switch or Rastelli procedure, the stronger morphologic left ventricle (mLV) is restored to systemic duty. However, since the mLV has lived a low-pressure life, it often needs “training” via pulmonary artery banding—no pain, no gain. Though technically demanding and sometimes staged, the double switch offers better long-term survival, improved ventricular function, and fewer regrets. In ccTGA surgery, the moral is clear: “Putting the right ventricle (mRV) in the right place at the right time is like hitting the iron while it is hot - A place for everything, and everything in its place”.[4] Anzai et al. did a meta-analysis, totaling 44 studies consisting of 1857 patients which included 535 patients with physiologic repair and 1322 patients with anatomical repair (atrial-arterial switch and atrial switch–Rastelli).[5] They analyzed the composite outcome of all-cause death or transplant over a 10-year postoperative follow-up. In the anatomical repair group, there was less post discharge mortality (9.7% vs. 6.1%), lower reoperation rates (20.6% vs. 17.9%), and less ventricular dysfunction (43% vs. 16%). However, the in-hospital mortality rates were the same in both groups [Figure 1]. They noted better composite outcomes in those with a true arterial double switch technique, suggesting a protective benefit of favoring anatomic repair over physiologic repair.[5]Figure 1: Anzai et al from this meta-analysis (44 total studies, n = 1857), show that anatomic repair patient group had significantly less post-discharge mortality, lower reoperation rates, less postoperative ventricular dysfunction as compared to physiologic repair groupJacob et al. did a meta-analysis of 47 studies consisting of 1222 patients with physiologic repair and 1622 patients with anatomical repair. Also included were patients who underwent atrial-arterial switch and atrial switch–ventricular operations [Figure 2]. The study showed lower risk of mortality (17.4% vs. 11.7%) and reintervention at 10 years (30.3% vs. 24.5%) post-operatively over a period of 20 years in the anatomical repair group.[6]Figure 2: Jacob et al in this meta-analysis (47 studies, n = 2,844), found that composite of all-cause mortality or heart transplantation during hospitalization after the main index operation as well as reintervention risk at 10 years were less in the anatomically repaired patients who had surgery at <5 years of age and / or had preoperative pulmonary artery banding as compared to physiologic repair groupJacob et al. also noted that a Rastelli double switch technique carried no difference in mortality. But true arterial double switch was associated with overall lower reoperation rates, and anatomical repair done prior to 5 years of age and preoperative pulmonary artery banding were associated with increased survival.[6,7] It is interesting to note that sometimes the diagnosis of ccTGA is missed for a very long period of time. Alomrani et al. report a rare instance of coronary artery disease associated with CCTGA a 83 year old patient.[8] Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.