Our case reported on the rare association of cyanotic heart disease: tricuspid atresia

and transposition of great arteries in infant.

Cyanotic spell
'Tet spell' (also called 'hypoxic spell', 'cyanotic spell', 'hypercyanotic spell',
'paroxysmaldyspnea') is an episodic central cyanosis due to total occlusion of right ventricle
outflow in a patient with a congenital heart disease. Hypercyanotic spell characterized by
paroxysm of hyperpnea (rapid and deep respirations), irritability and prolonged cry,
increased cyanosis and decreased intensity of heart murmur1. If not treated in time it
may lead to limpness, seizures, neurological deficit and death. or a cyanotic spell, it may
seem reasonable to administer oxygen1.
Since the mechanism of hypoxemia is limited pulmonary blood flow and not
alveolar hypoxia, oxygen may be of limited benefit. The first maneuver to attempt is
the “knee-chest” position. This will increase systemic vascular resistance and decrease right
to left shunting at the VSD level. Intravenous fluid boluses of normal saline at 10 cc/kg may
increase right ventricular preload and therefore might increase right ventricular output. Care
should be taken, however, not to cause fluid overload. Also, correct any metabolic acidosis
that may be present. If the spell persists, then giving medications to calm the child may help.
Morphine maybe given to help with sedation but also with decreasing the respiratory
drive and therefore decrease patient’s hyperpnea which may be further exacerbating the
spell. If the spell continues to persist, then increasing systemic vascular resistance with
phenylephrine or norepinephrine may be attempted2.
Tricuspid atresia
Tricuspid atresia is congenital absence of the right-sided atrio-ventricular (AV) valve,
with hypolpasia of the right ventricle. The features that are variable in tricuspid atresia are: 10
the realtion of teh great vessels, and 2) the presence or absence of obstruction to pulmonary
blood flow. Tricuspid atresia is most likely to be confused with Fallot's tetralogy but it must
also be differentiated from the other forms of congenital heart disease that present with
cyanosis without the signs of pulmonary hypertension. These include pulmonary stenosis
with reversal of shunt through a septal defect, Ebstein's disease, some forms of transposition
of the great vessels, some forms of persistent truncus arteriosus, and other more primitive
types of congenital heart disease such as single ventricle, common atrio-ventricular canal, and
biloculate and triloculate hearts.
Transposition of the great arteries
Transposition of the great arteries (TGA) is traditionally classified as a “conotruncal
heart defect”, implying that TGA evolves from abnormal development of the outflow tract
(OFT) of the embryonic heart. TGA is one of the most common and severe malformations of
the heart, accounting for 5% to 7% of all CHD.It is the 2nd most frequent cyanotic CHD, but
the most frequent CHD diagnosed in the neonatal period. Non-syndromic cases of complete
TGA principally occur in two variants: (1) in combination with normal (concordant) atrio-
ventricular connections, or (2) in combination with abnormal (discordant) atrio-ventricular
connections5.

1. Taksande A, Gautami V, Padhi S, Bakshi K (2009). Hypercyanotic Spell. JMGIMS,

Vol: 14, No 7-9
2. Yates MC and Rao PS (2013). Pediatric Cardiac Emergencies. Emergency Med, 3:6.
3. Patel M, MD, Overy DC, MD, Kozonis MC, MD, FACC, Lynne L. Hadley-Fowlkes,
BS (1987). Long-Term Survival in Tricuspid Atresia. JACC Vol. 9(2): 338-440.
4. Brown JW, Heath D, Morris TL, Whitaker W (1956). Tricuspid Atresia. Br Heart J
18(4): 499–518.
5. Al-Zahrani RS, Alharbi SH, Tuwaijri RMA, Alzomaili BT, Althubaiti A, and Yelbuz
TM . (2018).Transposition of the great arteries: A laterality defect in the group of
heterotaxy syndromes or an outflow tract malformation?. Ann Pediatr Cardiol,11(3):
237–249.