Better Late Than Never

We present a case of anatomic repair of dextro-transposition of the great arteries (d-TGA) with ventricular septal defect (VSD) in a 55-year-old man who presented with acute heart failure. This case highlights the importance of multimodal imaging and multidisciplinary involvement in developing a comprehensive surgical and medical plan for adults with congenital heart disease. We think this is the oldest reported patient undergoing anatomic surgical repair of d-TGA with VSD.

ranged from 70% to 80% on room air.His disease was classified as NYHA functional class III.He was on furosemide, spironolactone, and metoprolol succinate.He was not on angiotensin-converting enzyme (ACE) inhibitor, because its use was limited by hypotension.
His presenting vital signs were as follows: heart rate 104 beats/min, blood pressure 96/54 mm Hg, and oxygen saturation 76% on room air.His admission physical examination was notable for jugular venous distension, IV/VI systolic ejection murmur at the left Once euvolemic, he underwent right and left heart catheterization, which revealed normal pulmonary vascular resistance (PVR) and mildly elevated filling pressures (Table 1).

MANAGEMENT (MEDICAL/INTERVENTIONS)
Presenting in decompensated heart failure made anatomic surgical repair prohibitively high-risk.He      RV-PA conduit, potential for residual VSD, and baffle obstruction. 3In addition, these patients are at higher risk of biventricular dysfunction, arrhythmias, and sudden death.Access to an ACHD center is important for these patients for both treatment decisions and follow-up and is associated with a reduction in mortality that is more significant for patients with more complex disease. 4Our patient was seen at non-ACHD centers and told on multiple occasions that surgical repair was not feasible.His distance from an ACHD center served as one barrier to receiving a more complete evaluation earlier in life.Those living a greater distance from an ACHD center face longer travel, making it more difficult to schedule appointments, and tend to have more sociodemographic disparities. 5These factors led to a delay in his repair until age 55 years, whereas most Rastelli procedures are performed between the ages of 6 months and 40 years, with 60% being completed before the age of 5 years. 6We think our case documents the oldest anatomic repair for d-TGA/VSD.

FOLLOW-UP
He was seen in the ACHD clinic 1 month, and most

FUNDING SUPPORT AND AUTHOR DISCLOSURES
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
ADDRESS FOR CORRESPONDENCE: Dr Wilson Lam, One Baylor Plaza, Baylor College of Medicine, Houston, Texas 77030, USA.E-mail: wlam@bcm.edu.Adult Repair of d-TGA

Figure 3 ,
upper sternal border with radiation to the lung fields, III/VI decrescendo diastolic murmur at the left lower LEARNING OBJECTIVES To review the invasive and noninvasive workup of d-TGA with VSD.To understand what definitive surgical operations exist to treat d-TGA.To understand the importance of ACHDspecific care in adults with congenital heart disease.ISSN 2666-0849 https://doi.org/10.1016/j.jaccas.2024.102269sternal border, bounding pulses, and a sternotomy scar.Other pertinent findings included diminished breath sounds in the bilateral bases, hepatomegaly, cyanosis, clubbing, and lower extremity edema with stasis changes (Figure 1).He was admitted to the hospital in acute decompensated heart failure.PAST MEDICAL/SURGICAL HISTORY Past medical history included d-TGA and VSD diagnosed after birth.He underwent Blalock-Hanlon atrial septectomy at the age of 18 days.INVESTIGATIONS The patient's electrocardiogram showed firstdegree atrioventricular block (PR interval 230 ms), interventricular conduction delay (QRS duration 114 ms), and prolonged QTc (503 ms).Echocardiography revealed a subpulmonic left ventricular ejection fraction (LVEF) of 50% and a systemic right ventricular ejection fraction (RVEF) of 40%, significant subvalvar and valvar pulmonary stenosis (inadequate spectral Doppler to fully quantify), severe aortic regurgitation, severe aortic root dilation (6.0 cm in maximum dimension), and a large unrestrictive inlet VSD (Figure 2).Cardiac magnetic resonance imaging (MRI) showed interatrial communication, an inlet VSD, an enlarged left ventricle with LVEF 46%, flattening of the interventricular septum, significant right ventricular enlargement, and concentric hypertrophy with RVEF 31%, PS, aortic root dilation (6.0 Â 5.9 cm), and significant aortic regurgitation (regurgitant fraction 64%).Computed tomographic angiography (CTA) showed aneurysmal dilation of the left anterior descending artery at the branching of the first diagonal with mild ectasia of the right coronary artery (Video 1).

FIGURE 1
FIGURE 1 Cardiac Cachexia and Stasis Dermatitis With Subsequent Resolution

FIGURE 2
FIGURE 2 Transesophageal Echocardiography With Severe Pulmonary Stenosis

FIGURE 3
FIGURE 3 Computed Tomographic Angiography Depicting Variant Coronary Anatomy

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A C C : C A S E R E P O R T S , V O L . 2 Adult Repair of d-TGA RVEF 31%.Postoperative LVEF was 46% and RVEF only mildly depressed after the surgery.DISCUSSIONWe present a rare case of d-TGA, VSD, and PS presenting in an older male as decompensated heart failure.Diagnostic work-up revealed aortic root dilation complicated by severe aortic regurgitation treated with definitive repair.This case highlights the importance of multidisciplinary care in managing complex adult congenital heart conditions.In patients with unrepaired isolated d-TGA, the mortality rate is 89% at 12 months.1In comparison, in patients with a concurrent VSD the average life expectancy is 22 months.1It is rare for a patient to survive over 50 years.In this case it was likely possible because of his large VSD, surgically created atrial connection, and his pulmonary stenosis, which limited pulmonary overcirculation (Qp:Qs 2:1) and created favorable pulmonary artery pressures.However, this case highlights the importance of ACHD specialists evaluating these patients before development of the sequelae seen in this case.Surgical correction for patients with d-TGA, VSD, and PS most commonly consists of the Rastelli procedure, involving baffling of the VSD from the left ventricle to the aorta and creation of a conduit from the right ventricle (RV) to pulmonary artery (PA).

FIGURE 4
FIGURE 4 Modified Mullins Diagram Depicting Surgical Steps recently 3 years, after surgery with a favorable postoperative course.He weaned off all medications (except warfarin for his mechanical valve), with restoration of normal heart size and function.Also, despite the lack of Maze procedure, permanent pacing, or antiarrhythmic medication, he remains in sinus rhythm.This demonstrates that volume unloading, improvement in cardiac pressure, and restoration of normal saturations can be sufficient to address atrial arrhythmias in the correct clinical context.He remains physically active and engaged in house projects without cardiac symptoms, consistent with NYHA functional class I. CONCLUSIONS Complex congenital heart defects require long-term surveillance at a specialized ACHD center.Multimodality imaging and multidisciplinary input is key to developing treatment plans.We think this is the oldest anatomic repair for d-TGA with VSD, at 55 years, facilitated by our multidisciplinary approach and made possible by protected PVR.

TABLE 1
Pressure Data From Right-and Left-Side