The determination to undertake closure of the PDA, modified Maze with radiofrequency ablation, left atrial reduction plasty, mechanical mitral valve replacement (MVR), mechanical aortic valve replacement (AVR), closure of the patent foramen ovale (PFO), ascending aorta external wrapping plasty, and temporary epicardial pacing lead implantation was predicated on several critical considerations:

$\bullet$ Comprehensive imaging findings: Echocardiography disclosed a PDA with a left-to-right shunt at the arterial level, severe mitral regurgitation, mild aortic regurgitation, dilatation of the ascending aorta, enlargement of the aorta and bilateral pulmonary arteries, left-sided chamber hypertrophy, and a modest pericardial effusion.

$\bullet$ Valve strategy and guideline support: The patient exhibited severe valvular heart disease complicated by persistent atrial fibrillation. In patients 65 years of age requiring valve replacement, the use of mechanical prostheses is recommended by the 2020 American College of Cardiology/American Heart Association (ACC/AHA) Guideline for the Management of Patients With Valvular Heart Disease (Class I, Level of Evidence B-NR) [10].

$\bullet$ Indication for AVR despite “mild” aortic regurgitation (AR): Although preoperative AR was graded mild, intraoperative assessment identified a structurally abnormal, thickened aortic valve with reduced leaflet pliability. Given the need for a mechanical MVR (and thus lifelong anticoagulation) and the extent of concomitant procedures, concomitant AVR was selected to avoid a future high-risk re-sternotomy should regurgitation progress, with minimal incremental risk once on cardiopulmonary bypass (CPB) and the aorta opened.

$\bullet$ Aortic remodeling plan: Quantitative imaging demonstrated a normal aortic root (annulus 24 mm, sinus 30 mm, sinotubular junction 30 mm) with isolated dilatation of the proximal ascending aorta to 41 mm. Intraoperative inspection confirmed an intact root without coronary pathology. Accordingly, external wrapping plasty of the ascending aorta was performed using a #28 synthetic graft; the root was left intact and no coronary reimplantation was undertaken (non-Bentall).

$\bullet$ Single-stage approach: A single-stage operation was chosen to address the congenital lesion and multi-valvular/atrial pathology while avoiding re-sternotomy. Performing PDA closure, MVR/AVR, Maze, PFO closure, and ascending aorta plasty within the same CPB session was expected to reduce cumulative risk relative to staged procedures and repeat anesthesia.

$\bullet$ Access and hemostasis considerations: The necessity for meticulous hemostasis at multiple incision and reconstruction sites under direct visualization was a principal reason to forgo a minimally invasive approach.

$\bullet$ Postoperative rhythm management: Given the heightened risk of brady-arrhythmia after atrial fibrillation (AF) surgery and atrial remodeling, temporary epicardial pacing was instituted as a precautionary measure.

After the administration of adequate anesthesia, the patient is positioned in the supine position, followed by routine skin antisepsis and draping. The right femoral artery is exposed for subsequent cannulation. A median sternotomy is performed, and the pericardium is opened, revealing mild pericardial effusion with a pale yellow hue, alongside significant cardiac enlargement, primarily affecting the left atrium and ventricle. The aortic-to-pulmonary artery ratio is 1:1, with dilation of the aortic sinus and ascending aorta. Palpable tremors are noted at the apex and aortic root. The left superior vena cava is absent.

After administering heparin, extracorporeal circulation is established, ensuring an activated clotting time (ACT) greater than 480 seconds while maintaining systemic perfusion. With the heart beating, the PDA is exposed through the pericardial reflection between the distal main pulmonary artery and the descending aorta. Under continuous transesophageal echocardiographic (TEE) color-Doppler guidance, the duct is circumferentially mobilized using a right-angle clamp and blunt dissection, taking care to avoid injury to the recurrent laryngeal nerve and to protect the calcified pulmonary arterial end. Vessel loops are passed around both the aortic and pulmonary ends to permit a brief test occlusion while systemic arterial and pulmonary artery pressures are monitored; abolition of the continuous shunt signal on TEE confirms effective interruption. Definitive closure is then achieved by double-end ligation (2-0 silk ties buttressed with felt), followed by over-sewing of the pulmonary arterial stump with a continuous 4-0 polypropylene suture to address the calcified edge. Fibrin sealant is applied to the suture line, and final TEE demonstrates no residual color-Doppler flow across the duct. Bipolar clamp ablation is subsequently performed on the right atrium.

Cooling is initiated, and ventricular fibrillation is induced. The ascending aorta and both the superior and inferior vena cavae are then occluded. The aortic root is incised, allowing for coronary perfusion with oxygenated blood and myocardial protection solution. The interatrial septum is opened, and the oval foramen is found to be patent. The patent foramen ovale was primarily closed with 4-0 polypropylene sutures. The mitral valve is thickened, with shortened and constricted chordae tendineae, resulting in severe stenosis and incompetence. The aortic valve is mildly thickened, without commissural fusion, and exhibits mild incompetence. Both the mitral and aortic valves are excised, retaining a portion of the posterior mitral leaflet and chordae tendineae.

The left atrial appendage is excised, and left atrial ablation is performed. Partial resection of the posterior wall and roof of the left atrium is followed by left atrial plasty.

A mitral valve sizer (#31) and an aortic valve sizer (#25) are used to determine the appropriate sizes. The #31 Chinese mechanical mitral valve is implanted with interrupted 2-0 polyester sutures. After knotting, the valve opens and closes as expected. A portion of the interatrial septum is excised, and the heart cavity is irrigated with saline. The septum is then sutured with 4-0 Prolene sutures, and after lung deflation in the head-down position, the knots are secured.

The tricuspid valve is assessed by inserting three fingers, revealing mild regurgitation, which does not require intervention. The #25 Chinese mechanical aortic valve is implanted using continuous 2-0 Prolene sutures, and after knotting, the valve function is confirmed.

The aortic incision is closed with continuous 5-0 Prolene sutures. Following lung deflation in the head-down position, the knot is tied, and the patient is rewarmed. A defibrillation shock (20 J) is delivered, restoring normal sinus rhythm, as confirmed by ECG.

An external wrapping plasty of the ascending aorta is performed using a #28 synthetic graft; the aortic root remains intact and no coronary reimplantation is undertaken, therefore this is not a Bentall procedure.

Extracorporeal circulation is discontinued, blood is returned, and heparin is neutralized with protamine sulfate. All cannulation tubes are removed without complication. Bleeding is noted at the junction of the aortic root and left atrial roof. Five interrupted sutures fail to control the bleeding, and an autologous pericardial patch is used to cover the area, with drainage directed into the right atrium. A temporary pacing wire is placed on the epicardium to ensure hemostasis. Three mediastinal drainage tubes are inserted, and the sternum is closed with wire.

After confirming that all instruments and sponges are accounted for, the chest is closed in layers.

On the first postoperative day following complex corrective cardiac surgery, the patient developed acute kidney injury, hepatic dysfunction, circulatory shock, and postoperative sepsis, indicative of severe multiple organ dysfunction (Table 2). Continuous renal replacement therapy (CRRT) was initiated to maintain fluid and electrolyte balance, as well as to mitigate the accumulation of uremic toxins. Hepatoprotective treatment, including reduced glutathione and polyene phosphatidylcholine, was administered to alleviate hepatocellular damage.

Hemodynamic instability was managed with escalating vasopressor support, utilizing dopamine, norepinephrine, and epinephrine, titrated to maintain the target mean arterial pressure. Empirical antimicrobial therapy with piperacillin-tazobactam was commenced, and subsequently escalated to imipenem upon microbiological confirmation of Klebsiella pneumoniae infection. Additional supportive measures included transfusions of leukocyte-depleted red blood cells and fresh frozen plasma to correct anemia and coagulopathy, alongside continuous pericardial and mediastinal drainage to prevent cardiac tamponade.

During the early postoperative period, the patient experienced supraventricular tachycardia that was refractory to pharmacological cardioversion with amiodarone. As a result, synchronized direct-current cardioversion was performed. This multidisciplinary, multimodal management approach aimed to stabilize hemodynamics, halt the progression of organ dysfunction, and reduce perioperative mortality risk, particularly given the high-risk nature of the patient’s congenital and acquired structural heart disease.

At 1-year follow-up, transthoracic echocardiography demonstrated a pronounced reduction in cardiac volume overload with substantial regression of left atrial and ventricular dilation: left atrial diameter decreased from 89 mm preoperatively to 61 mm, and LVEDD from 88 mm to 63 mm. LVEF improved from 60% to 70%, and pulmonary artery systolic pressure remained consistently below baseline values, confirming effective elimination of the left-to-right shunt and restoration of cardiac function (Fig. 2). The NYHA functional class improved from III before surgery to I, with the patient able to undertake normal daily and moderate physical activities without limitation; palpitations and exertional dyspnoea had largely resolved, and exercise tolerance was markedly enhanced.

Fig. 2.

TTE in the first year after surgery. (A) The left heart is enlarged, but it is better than before. (B) Heart function is restored.

Between the second and third postoperative years, cardiac chamber dimensions and function remained stable, with LVEF maintained at about 60% and PASP persistently below preoperative levels. NYHA functional class remained at I, and there were no arrhythmia-related symptoms or signs of heart failure.

By the fourth postoperative year, LVEF was preserved at 60%, although mild mitral regurgitation (vena contracta 5 mm) was detected and judged to be clinically insignificant. At the 5-year review, echocardiography continued to show stable cardiac structure and function, with mild mitral regurgitation and PASP within non-significant ranges. No further atrial or ventricular enlargement was observed, indicating durable haemodynamic correction.

Throughout the entire follow-up period, the patient remained in NYHA class I, continued oral warfarin and metoprolol therapy, and reported no restrictions in occupational or social activities. These findings indicate that PDA closure in this context provided sustained improvement in cardiac structure and function, without major late complications, over a 5-year period.

At the most recent visit, on June 6, 2025, the patient reported that daily life and work remained unaffected. Occasional palpitations and chest tightness occurred only during vigorous exertion, resolving with rest. He continued anticoagulation and beta-blocker therapy, and his clinical condition remained stable.