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No full textAn equivalent posture to squatting is seen in an unoperated adult with tetralogy of Fallot.
No full textSir,
The advances in paediatric cardiology and cardiac
surgery have led to earlier and earlier surgical repair
of congenital cardiac malformations. In consequence,
the clinical picture of many congenital
cardiac malformations has changed, and some
signs and symptoms are no longer observed in their
typical appearance. Sometimes, however, it is
possible to recognise the transformation of an old
symptom or sign into a new one, with a new clinical
appearance, as we have recenrly observed in an adult
patient with retralogy of Fal1ot.
The old sign is squarring, which is the
characteristic instinctive knees-to-chest-posirion
assumed after exertion by patients with certain
rypes of cyanotic congenital cardiac malformations,
notably tetralogy of Fallot. Squatring was initially
brought to prominence by Taussig,' and generally
appears at about one year of age, or when the child
starts waiking. Investigarions have shown that the
saturations of oxygen, diminished by exercise, can
return to the levels prior ro exercise more rapidly
when the subject adopts a squarring rather than
a standing position.''' This beneficial effect of
squatting is probably due to two phenomenons.
The first is the exclusion from the circulation of the
highly unsaturated blood from the legs, due to the
compression of the femoral vessels. The second is
the reduction in the degree of right-to-left shunting
due to the increase ofperipheral vascular resisrance,
which opposes the right-to-left shunt through the
ventricular septal defect, and increases the flow of
blood to the 1ungs. During hypoxic spells, an infant
Correspondence to: Dr. Nicola Carano, Department of Pediatrics, Pediarric
Cardiology Unit, University of Parma, Via Gramsci 14, 43lOO parma, Italy.
Tel : * 390) 2 1 7021 42; Fax: + 190121,1 02394t E-mail: [email protected]
incapable of standing also tries to obtain the same
physiological effects of squatting, compressing his
abdomen against the shoulder of his caregiver.
1ù7hat happens in adults? \We have recently
observed a 40 year-old man with tetralogy of Fallot,
who had always refirsed surgicai rreatmenr. His
clinical condition progressively worsened during his
life, with increasing cyanosis, dyspnoea on exertion,
and severe limitation in his physical activity. This man
previously loved working in the countryside, and
would not abandon this hobby even if it caused him
severe fatigue and cyanosis. §7hen he was exhausted,
he would return home on his tractor, and press his
abdomen against the top of a small refrigeraror that
was in the garage, assuming a position with his legs
on one side of the refrigerator and his head on rhe
other. This position permitted him more rapidly to
return to his usual srate more rapidly. This clinical
observation indicates that adults with retralogy of
Fallot use manoeuvres that are equivalent to squarring
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