Microsoft word - tof case study--no answers

Advances in Quality and Outcomes; An STS National Database Event
September 29, 2005
Congenital Heart Surgery Database

Case Study: TOF

10 mos old Hispanic female referred for definitive repair. Birth history: Born at 30wk gestation
weighing 3.2kg. Shortly after birth patient became tachypneic and cyanotic with oxygen saturations in the
60’s. An initial echo showed a malalignment VSD with overriding aorta, functional pulmonary valve atresia,
normal coronary arteries, moderate PFO, main and branch PAs present and confluent, left aortic arch, and
PDA. No antenatal diagnosis had been made.
Neonatal history: On day 2 of life pt had an episode of hypotension and bradycardia resulting in a
cardiac arrest. Resuscitative efforts lasted for 35mins and pt was revived. On day of life 3 pt had a head
ultrasound which showed no hemorrhage or infarct but did demonstrate agenesis of the corpus collosum.
On day of life 4 pt underwent palliative surgery which consisted of a right Blalock Taussig shunt (rBTS) and
PDA ligation performed through a median sternotomy, off pump. The remainder of the patient’s hospital
course was complicated by line sepsis, rule out necrotizing enterocolitis and feeding issues. The pt, however,
did go home at 6weeks of age with oxygen saturations in the 80’s on room air (RA) on a medication regimen
of aspirin, lasix and digoxin. Pt was taking po ad lib feeds without difficulties and showed no neurological
sequelae.
Current history: Clinically, since her shunt, the pt has remained remarkably stable. Significant findings
have been a slow, progressive decline in oxygen saturations from high 80’s to presently low 80’s – mid 70’s on
RA. Her physical exam has also been remarkable for softening of her right ventricular outflow murmur. Her
current weight and height at 10mos. are 7.5kg and 69cm respectively. Most recent echo reveals a mid-septal
PFO with trivial left to right shunting, a large perimembranous, malaligned VSD resulting in severe, infundibular obstruction,
underdeveloped pulmonary valve annulus between 4-5mm, thickened pulmonary valve leaflets with antegrade flow in excess of
4m/sec (~64 torr). Aortic arch is left-sided, and unobstructed, coronaries are normal. Trace aortic insufficiency. Patent rBTS.
Good biventricular systolic function.
Cardiac catheterization at 9 mos of age: TOF/severe pulmonary stenosis (PS) with significant long-
segment right ventricular outflow tract (RVOT) obstruction with very little demonstrable antegrade flow
across the RVOT. Marked hypoplasia of the pulmonary valve annulus and proximal main pulmonary artery.
Branch pulmonary arteries were confluent and well developed. Shunt was patent with a mild degree of distal
narrowing. Left aortic arch and patent foramen ovale.
Pt was admitted for definitive repair at 10mos of age (Oct 14). Preoperatively the pt was on Aspirin. In
addition the pt. had been started on Reglan and Zantac for reflux. Oxygen saturations at time of preop exam
were 68% on RA. In the OR the pt underwent complete repair consisting of takedown of the rBTS,
pericardial patch closure of the VSD, infundibulectomy, pulmonary valvectomy, pericardial patch
reconstruction of the RVOT with a transannular patch (TAP), and suture closure of the PFO, performed
through a median re-sternotomy. Pt underwent bi-caval cannulation and was placed on cardiopulmonary
bypass (CPB) with cooling to 28oC. Total bypass time was 73minutes, cross clamp time was 50minutes. The
chest was closed in the usual manner.
Pt was initially intubated at the beginning of the case at 0922. Following surgery pt was transported to
the Cardiothoracic intensive care unit (CTICU) intubated, on Milrinone and Dopamine, with stable vital signs
(VS) and a normal sinus rhythm. On post operative day (POD) #1 (Oct 15), pt was extubated at 12 noon
and placed on 1L nasal cannula. Chest tube and foley were removed. On POD #2 pt was transferred to the
ward. On POD #3 pt was weaned to RA with good po intake and stable VS. Pacing wires were removed.
POD #5 pt remained hemodynamically stable with clear CXR and saturations 90-99% on RA. Pt was
discharged home on Lasix, Digoxin, Motrin, and Tylenol w/Codeine. Discharge diet was regular for age. Pt
was seen in post-op cardiothoracic clinic 3 days after discharge and was alert and happy. Of note pt had
redness and swelling at the superior aspect of the sternal wound and was started on a 10 day course of po
Keflex, pt was afebrile with stable VS. On follow-up exam after antibiotics - sternal wound was clean, dry and
intact without signs of infection.

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