Submitted by Dr Ian Chan, ICU, United Christian Hospital, Hong Kong; 24 Jan 2010
A 20-year old gentlemen with history of Down’s Syndrome, hypothyroidism, obstructive sleep apnoea and corrected Tetralogy of Fallot at 5 years old (1995) was admitted to the Intensive Care Unit because of Human Swine Influenza pneumonia.
Echocardiogram in 2004: dilated RV, TI ~ 25mmHg, RVOT patent, mild turbulence in MPA, PG ~ 7.4mmHg, PI, No residual VSD, mild AI, LVEDD ~4.07cm, LVFS 31%
His father has noticed cyanotic spells, particularly when the patient was suffering from respiratory tract infection or tonsillitis, since several years ago.
Mr. M was suffering from severe respiratory failure with refractory desaturation after admission, which was not responsive to high ventilatory support and FiO2 1.0.
Echocardiogram was repeated with agitated saline.
What is the diagnosis?