If you don't remember your password, you can reset it by entering your email address and clicking the Reset Password button. You will then receive an email that contains a secure link for resetting your password
If the address matches a valid account an email will be sent to __email__ with instructions for resetting your password
Address correspondence to Dr. Madan Mohan Maddali, Senior Consultant in Anesthesia, National Heart Center, Royal Hospital, P.B.No: 1331, P.C: 111, Seeb, Muscat, Sultanate of Oman
A 6-MONTH-old boy, (weight: 4.8 kg, height: 60 cm), diagnosed with pulmonary atresia
and intact ventricular septum, was taken to the operating room for an intracardiac
repair. The child previously had undergone pulmonary valve perforation and balloon
valvuloplasty. The preoperative transthoracic echocardiogram displayed 2 balanced
ventricles and a right ventricle composed of a sinus (inlet) portion, a trabecular
part, and a conus (infundibulum). The ventricular septum was intact. A secundum atrial
septal defect was present. There was mild tricuspid regurgitation. The right ventricular
systolic pressure was 80 mmHg to a systemic systolic arterial pressure of 70 mmHg.
The right ventricular outflow tract was severely narrowed, measuring 3.5 mm in diameter.
The pulmonary valve was doming, and the annulus was 7.8 mm. The pressure gradient
across the right ventricular outflow tract was 82 mmHg. Intraoperatively, resection
of the muscular right ventricular outflow tract with reconstruction of the tract using
a bovine patch was performed. A pulmonary valvotomy also was completed. Subsequently,
the right ventricular pressure was half of the systemic arterial pressure, by invasive
measurement, with a 10-mmHg pressure gradient across the pulmonary valve. The child
easily separated from cardiopulmonary bypass on milrinone (0.5 µg/kg/min). He was
taken to the intensive care unit on mechanical ventilation with stable hemodynamics.
On admission to the intensive care unit, the arterial oxygen saturation was >95%,
with an inspired oxygen concentration of 60%. Over the next 6 hours, he developed
arterial desaturation. The child was treated with a combined ventilation mode of synchronized
intermittent mandatory ventilation with pressure control and pressure support, positive
end-expiratory pressure of 6 cmH2O, and a fraction of inspired oxygen of 0.8. Transthoracic echocardiography showed
the shunt across the atrial septal defect (Fig 1, A and B; Videos 1 and 2). Color-flow Doppler of the hepatic veins showed a reversal of flow corresponding
to atrial systole (Fig 2; Video 3). The tricuspid annular plane systolic excursion was 0.627 cm, and the E/A ratio
was 0.9 (Fig 3, A and B). The color Doppler blood flow of the right ventricular outflow tract showed
flow turbulence beginning below the pulmonary valve, with a peak gradient of 22 mmHg
and mild pulmonary regurgitation (Fig 4; Video 4). Right ventricular outflow tract interrogation with continuous-wave Doppler revealed
pandiastolic (peak velocity: 84.8 cm/s) antegrade flow toward the pulmonary artery
(Fig 5, white arrows). Agitated saline injected into the right internal jugular vein was
seen in the atrium and the ventricle, displaying the shunt's nature (Fig 6; Video 5). What is the diagnosis?
Fig 1A subcostal 4-chamber transthoracic echocardiography view displaying a right-to-left
(A), and a left-to-right (B) shunt across the atrial septal defect.
Fig 5Continuous-wave Doppler interrogation of the right ventricular outflow tract showing
pandiastolic antegrade toward the pulmonary artery (white arrows).
Antegrade diastolic pulmonary arterial flow as a marker of right ventricular restriction after complete repair of pulmonary atresia with intact septum and critical pulmonary valve stenosis.
Residual restrictive right ventricular physiology after one-and-a-half ventricular repair conversion in pulmonary atresia with intact ventricular septum.
Right ventricular diastolic function 15 to 35 years after repair of tetralogy of Fallot. Restrictive physiology predicts superior exercise performance.
Cardiorespiratory responses to negative pressure ventilation after tetralogy of Fallot repair: A hemodynamic tool for patients with a low-output state.
