Abstract
The prognosis of tetralogy of Fallot (TOF) has improved in recent years, but complications in the late postoperative period remain a serious problem. These complications, combined with specific hemodynamic and structural abnormalities, make it difficult to determine the optimal treatment plan. A man in his early 60s had been diagnosed with TOF in his early teens; he had undergone ventricular septal defect closure and right ventricular outflow tract repair in his mid-20s. Approximately 40 years after surgery, he was referred to our hospital because of worsening heart failure due to moderate aortic regurgitation with left ventricular dysfunction caused by a residual ventricular septal defect, marked continuous right ventricular dilation and dysfunction, and severe pulmonary and tricuspid regurgitation. The patient had clearly missed the optimal time for surgery and had a high surgical risk score. After the patient had been provided sufficient information regarding treatment and risks, he underwent pulmonary and aortic valve replacement, tricuspid annuloplasty, and shunt closure. No obvious perioperative complications were observed, and the heart failure had remained stable for 4 years following reoperation. We report this complicated case of TOF repaired in adulthood with marked biventricular remodeling, associated with a residual shunt and progression of valvular disease.
Learning objective
Surgical interventions for the multiple complications that occur during the long-term course after TOF repair, particularly in the context of right ventricular remodeling, may be associated with risks. In the field of adult congenital heart disease, rather than applying a single cut-off to determine the optimal timing for intervention, clinicians should consider factors such as the patients’ age, sex, and other individual characteristics, paying particular attention to the hemodynamic status.
Introduction
The prognosis of tetralogy of Fallot (TOF) has improved remarkably with advances in intracardiac repair, surgical techniques, and perioperative management. Thus, postoperative survival times have increased, leading to a rise in the number of reoperations during the late postoperative period. However, heart failure can still develop in some cases if timely and appropriate treatment is not provided. Here, we present a case of refractory heart failure occurring more than 40 years after TOF repair (rTOF) in adulthood.
Patient’s background
The patient was a man in his early 60s who had been diagnosed with TOF in his early teens and had finally decided to undergo treatment for TOF in his mid-20s. He underwent a right ventricular (RV) outflow tract incision, resection of a thickened muscle bundle, and closure of the ventricular septal defect (VSD) with a patch while preserving the pulmonary valve. His condition stabilized and follow-up was discontinued. However, 25 years later, he developed paroxysmal atrial fibrillation (AF) and was treated with oral medications, including amiodarone and warfarin. An echocardiogram revealed a residual VSD, mild aortic regurgitation (AR), and moderate tricuspid regurgitation (TR), with no evidence of RV outflow tract stenosis. He received oral treatment with an angiotensin receptor blocker (ARB), a β-blocker, and a diuretic. During the next 7 years, the paroxysmal AF progressed to persistent AF, and his left ventricular ejection fraction gradually decreased to <50 %. Six years later, pulmonary regurgitation (PR) rapidly developed due to pulmonary valve prolapse, and severe TR was observed. His weight increased by approximately 17 kg due to leg edema and ascites. Levothyroxine sodium hydrate was initiated to treat hypothyroidism. Despite adding oral inotropes, increasing the diuretic dose, and performing abdominal paracentesis, his heart failure remained uncontrolled, and his BNP level increased from 100 to 900 pg/mL. He was referred to our hospital for surgical retreatment because of worsening heart failure with insufficient control by oral medications (azosemide 120 mg, spironolactone 25 mg, tolvaptan 30 mg, warfarin 1.75 mg, bisoprolol fumarate 1.25 mg, digoxin 0.0625 mg, and levothyroxine sodium hydrate 50 μg).
Physical findings
At admission, the patient’s heart rate was 85 bpm, blood pressure was 105/66 mmHg, and oxygen saturation was 97 %. On physical examination, the first heart sound was normal, the second heart sound was accentuated, and a 3/6 holosystolic murmur was noted at the fourth left intercostal space along the sternal border. Ascites and leg edema were also observed.
Laboratory findings
The results of the patient’s blood tests are shown in Table 1 . He had chronic hepatitis C viral infection, although his liver enzymes were normal. His Child–Pugh score was B while on warfarin therapy. Technetium-99 m-galactosyl serum albumin hepatic scintigraphy indicated mild to moderate liver dysfunction. Chest radiographs showed pulmonary congestion and enlargement of the main pulmonary artery (PA) and right atrium (RA) ( Fig. 1 A ). An electrocardiogram showed AF, right axis deviation, and a nonspecific intraventricular conduction disorder ( Fig. 1 B). Echocardiography revealed significant biventricular enlargement and systolic dysfunction without evidence of localized asynergy. In the short-axis view, a patch closure area was observed from the right coronary cusp to the noncoronary cusp, and there was shunt flow from around this area. In addition to LV–RA communication, moderate AR, mild mitral regurgitation, severe TR with a dilated tricuspid annulus, and severe PR with septal semilunar valve prolapse were observed ( Fig. 2 ). The inferior vena cava was expanded, and the TR pressure gradient was 65 mmHg. The results of right heart catheterization and magnetic resonance imaging (MRI) are shown in Table 1 . MRI revealed marked enlargement and dysfunction of both ventricles. The pulmonary–systemic flow ratio calculated by cardiac pool scintigraphy and cardiac MRI was 2.1 to 2.7. Computed tomography showed a dilated PA, moderate chronic liver damage, mild splenomegaly, and massive ascites.
| Parameter | Units | Before surgery | 1.5 years after surgery | Normal reference interval |
|---|---|---|---|---|
| Blood test | ||||
| WBC | /μL | 5510 | 5300 | 4800–9800 |
| RBC | /μL | 4,410,000 | 4,820,000 | 4,100,000–5,300,000 |
| Hb | g/dl | 11.2 | 15 | 14–18 |
| Ht | % | 36.1 | 43.5 | 130–350 |
| Plt | /μL | 164,000 | 190,000 | 130,000–320,000 |
| CRP | mg/dl | 0.19 | 0.08 | 0–0.15 |
| UN | mg/dl | 47 | 22.6 | 8.0–22 |
| Cre | mg/dl | 1.42 | 1.43 | 0.6–1.1 |
| UA | mg/dl | 8.1 | 5.6 | 3.2–7.7 |
| ALP | IU/L | 459 | 118 | 38–113 |
| γ-GTP | IU/L | 59 | 19 | 11–73 |
| AST | IU/L | 18 | 21 | 12–31.0 |
| ALT | IU/L | 12 | 12 | 8.0–40 |
| LD | IU/L | 328 | 226 | 124–222 |
| T-Bil | IU/L | 2.1 | 1.4 | 0.2–1.0 |
| TP | g/dl | 7.3 | 8.5 | 6.7–8.3 |
| Alb | g/dL | 4 | 4 | 4.0–5.0 |
| FT4 | ng/dl | 1.59 | 1.6 | 0.7–1.7 |
| TSH | μU/ml | 2.48 | 1.86 | 0.5–4.3 |
| BNP | pg/ml | 742 | 143 | <18.4 |
| Cardiac magnetic resonance imaging | ||||
| RVEF | % | 28 | 31 | 47–58 |
| RVEDVI | ml/m 2 | 173 | 71 | 57–108 |
| RVESVI | ml/m 2 | 124 | 49 | 20–49 |
| RAA | cm 2 | 29 | 23 | 19–24 |
| LVEDVI | ml/m 2 | 165 | 97 | 53–99 |
| LVEF | % | 33 | 48 | 53–74 |
| Cardiac catheterization | ||||
| RAP | mmHg | 12 | n/a | 2–7 |
| PAWP | mmHg | 14 | n/a | 4–12 |
| mPAP | mmHg | 22 | n/a | 9–19 |
| PVR | WU | 2.9 | n/a | 0.25–1.625 |
| CI | L/min/m 2 | 1.7 | n/a | 2.5–4.0 |
| LVEDP | mmHg | 15 | n/a | 5–12 |
| MAP | mmHg | 70 | n/a | 70–105 |
| SVR | WU | 20.7 | n/a | 8.75–20 |
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