CARDIOVASCULAR JOURNAL OF AFRICA • Vol 21, No 4, July/August 2010
AFRICA
231
management in adults with TOF are: fibrosed and stiff right
ventricle, a tendency to develop congestive heart failure and
bilateral pleural effusions, and the presence of major aorto-
pulmonary collateral arteries (MAPCAs). The progressive
narrowing of the initially mild pulmonary artery stenosis in our
patient undoubtedly contributed to her longevity and then dete-
rioration of her symptoms later in her life.
Post-operatively, these patients are at risk of developing
pulmonary oedema, because of increased pulmonary vascularity
and large MAPCAs and may require prolonged ventilation with
positive end-expiratory pressure (PEEP). Congestive cardiac
failure leads to the development of bilateral pleural effusions
that may appear multiple times and may take weeks to months to
resolve completely.
Although complete repair of TOF in the neonate is associated
with excellent intermediate-term survival,
4
the overall survival
of surgically treated adult patients with TOF is also acceptable.
The major benefit of complete repair in adults is their functional
improvement. On the other hand, late complications closely
related to chronic hypoxia, such as arrhythmia and ventricular
dysfunction, might require more careful follow up after the surgi-
cal correction.
5
There are a limited number of cases of patients who have
lived with untreated TOF until adulthood. This case serves as a
reminder of TOF as a differential diagnosis in cynotic patients
with congestive heart failure, and its unique manifestations and
management.
References
1. Yang X, Freeman LJ, Ross C. Unoperated tetralogy of Fallot: case report
of a natural survivor who died in his 73rd year; is it ever too late to oper-
ate?
Postgrad Med J
2005;
81
: 133–134.
2. Bertanou EG, Blackstone EH, Hazerlrig JB,
et al
. Life expectancy with-
out surgery in tetralogy of Fallot.
Am J Cardiol
1978;
42
: 458–466.
3. Higgins CB, Mulder RG. Tetralogy of Fallot in the adult.
Am J Cardiol
1972;
29
: 837–846.
4. Hirsch JC, Mosca RS, Bove EL. Complete repair of tetralogy of Fallot
in the neonate: results in the modern era.
Ann Surg
2000;
232
(4):
508–514.
5. Atik FA, Atik E, da Cunha CR, Caneo LF, Assad RS, Jatene MB,
et
al
. Long-term results of correction of tetralogy of Fallot in adulthood.
Cardiothorac Surg
2004;
25
(2): 250–255.
Fig. 6. Non-enhanced CT scan of the chest.
28 August – 1 September 2010 European Society of Cardiology
congress (ESC)
Stockholm, Sweden
11 September 2010
Cape Kidney Association’s annual
fundraiser
Peninsula Room, Kenilworth
Race Course, Cape Town, SA
Contact Molly Fabe on 021 448-0817
14–17 October 2010
Vascular Society of Southern Africa
congress (VASSA)
KwaMaritane, North West
Province, SA
13–17 November 2010
American Heart Association congress Chicago, Illinois, USA
27–31 May 2011
10th PASCAR congress
Kampala, Uganda
Watch these dates
