Ewha Med J.  2017 Apr;40(2):94-98. 10.12771/emj.2017.40.2.94.

A 75-Year-Old Natural Survivor with Uncorrected Tetralogy of Fallot Presenting with Hypoxic Spell

• Song HY
• Kang SY
• Lee OJ
• Kwak JH

• KMID: 2379505
• DOI: http://doi.org/10.12771/emj.2017.40.2.94

Abstract

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Only a few patients reach adulthood without surgical correction. Unrepaired TOF patients with mild to moderate right ventricular outflow tract (RVOT) obstruction may be clinically silent until adulthood. TOF with hypoxic spells present as periods of profound cyanosis that occur because of almost total RVOT obstruction. So, hypoxic spell typically occurs in a crying infant but is rare in an adult. In this report, we presented a case of a 75-year-old patient with uncorrected TOF presenting with hypoxic spell, consequent pulmonary hypertension and chronic heart failure. This is the oldest case of natural survivor with uncorrected TOF in Korea and the oldest patient presenting hypoxic spell worldwide.

Keyword

Tetralogy of Fallot; Hypoxia; Adult

MeSH Terms

Adult
Aged*
Anoxia
Crying
Cyanosis
Heart Defects, Congenital
Heart Failure
Humans
Hypertension, Pulmonary
Infant
Korea
Survivors*
Tetralogy of Fallot*

Figure

• 

  Fig. 1 Chest anteroposterior at presentation. It shows severe cardiomegaly, pulmonary edema and pleural effusion.

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  Fig. 2 Echocardiographic findage. They show (A) right ventricular outflow tract (RVOT) obstruction, (B) both atrial enlargement and right ventricular hypertrophy, (C) perimembranous ventricular septal defect with bidirectional shunt and overriding of aorta, and (D) the peak pressure gradient of tricuspid regurgitation 115.83 mmHg. RV, right ventricle; RA, right atrium; LV, left ventricle; LA, left atrium; Ao, aorta.

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  Fig. 3 Echocardiography during extremely resting state. It reveals (A) relatively mild stenosis of the right ventricular outflow tract and (B) bidirectional shunt but predominantly left-to-right through the ventricular septal defect.

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  Fig. 4 Echocardiography during hypoxic spell. It (A) more severe subvalvular pulmonary stenosis and (B) predominantly right-to-left shunt through the ventricular septal defect.

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