Cyanotic tetralogy of Fallot in a 77 year old man

Thomas, Simon; Bass, Pat F.; Pambakian, H.; Marigold, J H

doi:10.1136/pgmj.63.739.361

Cited by 22 publications

(17 citation statements)

References 2 publications

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“…4 The pulmonary annulus was 13 mm in this patient, comparable to that reported in the other published cases-(age 45, PVD 10 mm), 4 (age 77, PVD 9 mm). 5 A second common feature is that of LVH as seen in this patient (16 mm); presumably this acts by delaying of shunting from the right to left ventricle. 6 7 LVH may be a late development in the natural history of Fallot and any beneficial effect may not be seen until adult life.…”

Section: Discussionmentioning

confidence: 70%

Unoperated tetralogy of Fallot: case report of a natural survivor who died in his 73rd year; is it ever too late to operate?

Yang

Freeman

Ross

2005

Postgraduate Medical Journal

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The case history is described of a man in his 73rd year who was one of the oldest surviving patients with uncorrected tetralogy of Fallot (ToF) before succumbing with renal failure. Factors contributing to his longevity included small pulmonary arteries and presumed slow development of subpulmonary obstruction together with moderate concentric left ventricular hypertrophy-features previously seen in long term survivors. Less than 3% of all patients with uncorrected ToF survive beyond their 40s but late operative repair is still a valuable option. Practicalities of renal dialysis in the presence of an intracardiac shunt are considered.T etralogy of Fallot (ToF), first described in 1888, comprises an interventricular septal defect, right ventricular outflow tract obstruction, an overriding aorta, and right ventricular hypertrophy (RVH).1 It is the most common form of cyanotic congenital heart disease (10% of all cases).2 This case describes a man in his 73rd year who was one of the oldest surviving patients with an uncorrected ToF before succumbing with renal failure. We review the factors contributing to his longevity and consider whether surgery in his 66th year would have been appropriate. CASE PRESENTATIONThe patient, described as having a weak heart in childhood, never attended school or played sports. Diagnosis of ToF was not made until the age of 31 (1961) but he defaulted from further investigations. In 1996 (aged 66 years) he was reassessed while being investigated for proteinuria (urea = 5.7 mmol/l; Cr = 98 mmol/l). He was asymptomatic. He was mildly cyanosed and clubbed with pectus carinatum. Blood pressure was 140/80 mm Hg. He had an ejection systolic murmur in the pulmonary area. Echocardiography disclosed a subaortic VSD with a left to right shunt of 1.94 m/s, minimal aortic override (diameter 3.76 cm), small volume, hypertrophied well contracting LV, RVH, dilated RA, bicuspid pulmonary valve with a small pulmonary artery (1.4 cm), subpulmonary stenosis with an RVOT gradient of 112 mm Hg, and moderate PR. Electocardiography confirmed sinus rhythym, voltage criteria for RVH; RBBB with a QRS duration of 100 ms. Chest radiography showed a right aortic arch. Holter monitoring disclosed one asymptomatic 4 beat burst of SVT. Exercise testing terminated at four minutes because of fatigue (O 2 saturation fell from 91% to 80%). The decision was made for medical management rather than surgical correction in view of his advanced age, asymptomatic status, and personal wishes. At the age of 69 the patient complained of regular fast palpitations and associated dyspnoea. The echocardiogram showed severely hypertrophied and impaired RV function, huge RA (6.868.2 cm), normal LV dimensions with moderate concentric hypertrophy as before, and a dilated LA (5.82 cm). Creatinine was 118 mmol/l. He was treated with frusemide, spironolactone, amiodarone, and aspirin. His renal function deteriorated in his 72nd year (urea = 18.5 mmol/l; Cr = 174 mmol/l), and he needed thyroxine for amiodarone induced hypothyroidism. He was n...

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Section: Discussionmentioning

confidence: 70%

Unoperated tetralogy of Fallot: case report of a natural survivor who died in his 73rd year; is it ever too late to operate?

Yang

Freeman

Ross

2005

Postgraduate Medical Journal

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“…9 Thomas et al reported the case of a 77-year-old man who remained asymptomatic until death, and the diagnosis of congenital defect was done postmortem. 10 In the case of our patient, survival until 85 was due to favorable anatomy of the tetralogy, especially to the moderate to severe RVOT obstruction that slowed pulmonary hypertension development.…”

Section: Discussionmentioning

confidence: 66%

“…Thomas et al. reported the case of a 77‐year‐old man who remained asymptomatic until death, and the diagnosis of congenital defect was done postmortem 10 …”

Section: Discussionmentioning

confidence: 99%

Unrepaired Tetralogy of Fallot in an 85-Year-Old Man

Gorla¹,

Franzoni²,

Rosa³

et al. 2012

Congenit Heart Dis

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Tetralogy of Fallot is the most common cyanotic congenital heart defect and accounts for about 5% of all congenital cardiopathies. The definitive treatment modality for tetralogy of Fallot is reparative surgery, which is recommended to be performed by the time of diagnosis. Without surgical repair, most patients would die during their childhood. In the past, survival data indicated that 66% of persons with tetralogy of Fallot not surgically treated lived until the age of 1, 49% lived until the age of 3, and 24% lived until the age of 10. We now present a rare case of a man with unrepaired tetralogy of Fallot who survived until the age of 85. He presented to our emergency room for dyspnea and palpitations due to a new-onset high-frequency atrial fibrillation and acute heart failure; transthoracic echocardiography showed the presence of tetralogy of Fallot. By consulting the scientific literature, we can say that this is the second patient who survived more than 80 years without surgical intervention.

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“…Iga et al reported the case of a 61‐year‐old woman with tetralogy of Fallot and dextrocardia with situs inversus who was able to survive to this age because of unusually low systemic pressures and a proper balance between VSD and pulmonary stenosis 15 . Chin et al similarly reported the case of a 61‐year‐old man, 16 and Thomas et al reported the case of a 77‐year‐old man who was asymptomatic as an adult until the seventh decade when he developed biventricular failure 17 …”

Section: Discussionmentioning

confidence: 99%

“…15 Chin et al similarly reported the case of a 61-yearold man, 16 and Thomas et al reported the case of a 77-year-old man who was asymptomatic as an adult until the seventh decade when he developed biventricular failure. 17 Hokansen et al studied the published autopsy cases of patients with tetralogy of Fallot who died without any surgical treatment to determine the life expectancy of such persons. The survival data indicated that 66% of persons with tetralogy of Fallot not treated surgically live up to the age of 1 year, 49% up to the age of 3 years, and 24% up to the age 10 years, with the chance of survival significantly less when pulmonary atresia, rather than stenosis, is present.…”

Section: Discussionmentioning

confidence: 99%

Survival to the Age of 52 Years in a Man with Unrepaired Tetralogy of Fallot

Makaryus¹,

Aronov²,

Diamond³

et al. 2004

Echocardiography

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Tetralogy of Fallot is the most common cyanotic congenital heart defect during infancy. It is composed of a ventricular septal defect, an overriding aorta, obstruction of right ventricular outflow, and right ventricular hypertrophy. Most patients experience cyanosis at birth and die in childhood without surgical intervention. The rate of survival at 40 years without surgical correction is only 3%. We present the case of a man with tetralogy of Fallot who survived until the age of 52 years without surgical intervention.

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Cyanotic tetralogy of Fallot in a 77 year old man

Cited by 22 publications

References 2 publications

Unoperated tetralogy of Fallot: case report of a natural survivor who died in his 73rd year; is it ever too late to operate?

Unoperated tetralogy of Fallot: case report of a natural survivor who died in his 73rd year; is it ever too late to operate?

Unrepaired Tetralogy of Fallot in an 85-Year-Old Man

Survival to the Age of 52 Years in a Man with Unrepaired Tetralogy of Fallot

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