Longevity in the Tetralogy of Fallot

Meindok, H.

doi:10.1136/thx.19.1.12

Cited by 16 publications

(11 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Firstly, a hypoplastic pulmonary artery with slow development of subpulmonary obstruction. 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.…”

Section: Discussionsupporting

confidence: 88%

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

View full text Add to dashboard Cite

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...

show abstract

Section: Discussionsupporting

confidence: 88%

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

View full text Add to dashboard Cite

show abstract

“…Three main factors have been identified for the longetivity in natural survivors with unoperated tetralogy of Fallot. First is the small pulmonary artery with presumed slow development of subpulmonary obstruction 9. Another factor identified is that of left ventricular hypertrophy10 as seen in our patient who had a inter-ventricular wall thickness of 14 mm in diastole.…”

Section: Discussionmentioning

confidence: 64%

“…First is the small pulmonary artery with presumed slow development of subpulmonary obstruction. 9 Another factor identified is that of left ventricular hypertrophy 10 as seen in our patient who had a interventricular wall thickness of 14 mm in diastole. It is said that left ventricular hypertrophy acts by delaying the shunting of blood from the right to left ventricle.…”

Section: Discussionmentioning

confidence: 72%

Uncorrected Tetralogy of Fallot in a 25-Year Old Nigerian African

Ojji

Babalola

Falase

2011

Clin Med Insights Case Rep

View full text Add to dashboard Cite

show abstract

“…1,2 However, complications such as hemoptysis, cerebrovascular accidents (CVAs), cerebral abscesses, hypoxemia, cardiomyopathy, infective endocarditis, and renal failure occur with increasing age. 3 Although the presentation of TOF in adults is becoming a rarity in the Western world, it is not uncommon in Third World countries.…”

Section: Introductionmentioning

confidence: 99%