Truncus

Arteriosus
Post operative management

Mohammad Gamaleldeen
MBBCh,MSc.,MRCPCH
PCICU Registrar
 Natural History

• The outcome of TA without surgical repair is generally dismal, though

there are occasional reports of survivors with unoperated TA.

• Case series of uncorrected patients have reported an average age of

death of five weeks with only 15 percent surviving to the age of one
year [1].

• All patients who survive beyond the first year of life subsequently
develop severe pulmonary vascular obstructive disease (ie,
Eisenmenger syndrome) with profound cyanosis and functional impairment
[2,3].
 Truncus arteriosus surgery
evolution

Until the early 1970s, typically, patients died between

the age of a few weeks to six months.

Congestive heart failure owing to large pulmonary

blood flow and truncal valve regurgitation was the
major cause of death until innovative surgical
techniques were discovered.

In 1963, Herbert Sloan at the University of Michigan

completed the first repair using a nonvalved Herbert Sloan, MD, 1914 - 2013

conduit with long-term survival.

 Truncus arteriosus surgery
1967
evolution

The first successful surgical correction of truncus

arteriosus was performed in 1967 by McGoon, Rastelli,
and Ongley, who used a valved aortic allograft to establish
4

right ventricular–pulmonary artery continuity [4].

1976, Ebert used the valved

conduit in infants under six
months of age (University of
California, San Francisco).
Truncus arteriosus surgery
evolution

Aswan
Technique
Post-operative complications
Preoperative considerations:

• Different variants of pulmonary artery origins can be found distal to the

common outlet valve – which can be insufficient and stenotic to different
degrees.

• As coronary anomalies and malformations of the aortic arch may be present,

cardiac catheterization and/or MRI/CT angiography should be performed for precise
visualization of the anatomy.

• As there is frequently a microdeletion on chromosome 22, appropriate genetic

testing should be requested [7].
Post-operative complications
Post-operative complications

• Many of immediate postoperative complications after complete Truncus repair are typical
for neonatal cardiac surgery [8].

• Myocardial dysfunction and systemic inflammatory response after CPB should be

anticipated [8].

• Inotropes and vasopressors are usually required for the 1st 24 to 48 hrs post-op [9].

• Milrinone.

Refractory hypotension
Calcium gluconate infusion ECMO

DiGeorge $ [10] Hydrocortisone Maximum medical therapy

Relative adrenal insufficiency [11]

 PICU management:

Factors aggravating LCOS

• The degree of truncal valve insufficiency is a key determinant of the initial ICU
course [12].

• Patients who might require ECMO for LCOS are potentially unsupportable if there is
significant truncal valve insufficiency [12].

• Afterload reduction may be helpful to augment COP in case of truncal valve

insufficiency [12].

• RV filling pressure is generally necessary for adequate COP due to RV restrictive

physiology [12].

• A small atrial communication sometimes is left during surgery to augment the

systemic COP.
 PICU management:

Factors aggravating LCOS

• Injury of the left coronary is possible during separation of the pulmonary

arteries.

• Coronary artery compression by RV to PA conduit is possible.

• Inability to demonstrate flow in both coronaries in postoperative TEE,

particularly in the presence of regional wall abnormalities, should alert the ICU
team about myocardial ischemia [12].
 PICU management:

Analgosedation

• Care should be taken to avoid pulmonary

hypertensive crisis.

• Good analgesia and sedation with or without

muscle paralysis is always needed for the 1st
24 to 48 hrs.

• It is also recommended to minimize handling to

prevent irritability.
 PICU management:

Arrhythmia
• Truncus arteriosus repair is a high risk operation for the development of JET.

• Standard management includes:

1. cooling,

2. reduction of B-adrenergic stimulation

3. Overdrive pacing.

4. Electrolytes correction.

5. Anti arrhythmic medications.

Ventricular arrhythmia is less common post Truncus arteriosus repair and

if present it raises the possibility of coronary artery injury [13].
 PICU management:

Bleedin
g

• Meticulous monitoring for ICD output and hemodynamics should be performed

to avoid hypovolemia and hypoxia which are triggers for pulmonary
hypertensive crisis.

• Increased risk for CMV infection and GVHD, so irradiated and CMV
seronegative blood should be considered until genetic testing results are
known[14].
 PICU management:

Ventilation

• A ventilatory strategy to maintain inflation at FRC utilizing the lowest possible

MAP is desirable to avoid negative effects on the RV filling.

• Respiratory acidosis should be avoided due to it’s effects on the PVR.

• Cleft palate, upper and lower air way anomalies are relatively common in
DiGeorge $.

• Extubation only following improvement of the PHT situation [12].

 PICU management:

Truncus Arteriosus with interrupted arch

• Additional concerns related to the arch repair:

1. Residual arch obstruction “manifested by lower extremity pulses and blood

pressure gradient between upper and lower limbs”.

2. Multiple suture lines along the aortic arch entail a higher risk of bleeding.

3. The longer bypass, cross clamp and circulatory arrest times required for arch
reconstruction may exacerbate the systemic inflammatory response [12].
 PICU management:

Pulmonary hypertensive crisis

• CPB is known to induce a systemic inflammatory response causing endothelial

dysfunction.

• During CPB pulmonary circulation is excluded, so the lungs are hypoxic and ischemic
with the risk of reperfusion injury.

• Endothelial cell injury leads to a decrease in the output of nitric oxide.

• Lung injury is clinically manifested by reduced oxygenation and reduced lung

compliance.

• Patients with preexisting pulmonary vascular endothelial dysfunction are at greater risk
for developing CPB induced lung injury [16].
 PICU management:

Pulmonary hypertensive crisis

• Diagnosis:

• Patient may display signs of Rt. Side preload and RV failure with signs of sudden decrease
left side preload and LCOS.

• Clinical diagnosis is facilitated by the use of indwelling catheters allowing continuously

monitoring of pulmonary pressure and/or LA pressure.

• The patient may also have signs of ischemia due to reduction of coronary flow.

• Desaturation may happen in the presence of right to left shunt.

• Patients also may have arrhythmias, persistent hypoxia and metabolic acidosis [16].
 PICU management:

Pulmonary hypertensive crisis

• Diagnosis:

• Chest x rays are unspecific for diagnosis, but may be useful to rule out triggering
factors like volume overload and the presence of added pulmonary disease like
atelectasis, pneumothorax, hyperinflation, or pleural effusion.

• ECG may be useful in patients who develop secondary arrhythmia or ischemic

changes [16].
 PICU management:

Pulmonary hypertensive crisis

• Diagnosis:

• Echocardiography remains the cornerstone technique to rapidly assess pulmonary hypertension in the intensive
care setting.

• Measuring RV pressure by tricuspid regurgitation [17].

• IVS geometry.

• The presence of pulm. Regurgitation allows estimation of pulmonary diastolic and mean pressures.

• Diagnosis of interatrial and interventricular shunts.

• The presence of residual lesions which may be the origin of the spell.

• Follow up of the therapeutic efficiency.

After proper management
 PICU management:

Pulmonary hypertensive crisis

• Therapeutic measures: [18].

• 1st of all anatomical problems should be excluded as residual shunts and

outflow tract obstruction.

• Well controlled analgesia , sedation and if necessary muscle relaxants.

• Adequate ventilation is essential avoiding overdistension and atelectasis.

Relation between lung volume and PVR [19]
The effect of PH and PO2 on
PVR [20]
 PICU management:

Pulmonary hypertensive crisis

• Therapeutic measures: [21]

• Normal to slightly alkalotic PH. The use of sodium bicarbonate or THAM may be
considered in some patients to avoid deleterious effects of hyperventilation.

• Hyperventilation to induce alkalosis by low CO2 levels may cause an increase in

systemic vascular resistance that may be not tolerated.

It is the PH not
CO2
 PICU management:

Pulmonary hypertensive crisis

• Therapeutic measures: [22]

• Inhaled nitric oxide improves RV systolic function by decreasing it’s

afterload while increasing left ventricular preload restoring aortic pressure
and coronary perfusion.

• Patients with poor LV function it should be used cautiously.

• Many researches showed that even low dose iNO has a maintained effect
over several days.

• Patients who remain dependent on NO and have rebound PHT are

candidates to sildenafil during weaning.

• Inhaled prostacyclin is increasingly used, having the advantage of delivery

by aerosol without a special device [23].
 PICU management:

Pulmonary hypertensive crisis

• Therapeutic measures:

• Sildenafil is as effective as iNO as a pulmonary vasodilator.

• It can be used in the setting of iNO withdrawal. [24]

 PICU management:

Pulmonary hypertensive crisis

• Therapeutic measures:

• Inotropes and Vasopressors: [25]

• The perfect drug should improve myocardial performance and vasodilate the pulmonary
vascular bed without inducing tachycardia and increasing O2 consumption !.

• Most centers initiate Milrinone intraoperative, with a low dose of Epinephrine to support
function.

• Norepinephrine through an increase in SVR may improve coronary perfusion as such as RV

function.

• Some other therapies are still under research for use in acute PHT as Nesiritide and
Levosimendan.
PICU management:
 Conclusions:

• Current Truncus Arteriosus surgical repair techniques are still developing with better
short and long term outcomes. Preoperative assessment using different modalities is
crucial for predicting and avoiding postoperative complications.

• Early identification of the underlying cause of patient compromise can prevent

unwanted outcome.

• Pulmonary hypertension remains challenging with a significant mortality and

morbidity.

• Appropriate therapy firstly require proper identification of the cause.

• A multidisciplinary approach involving surgical and medical teams case by case is

important for achieving good results.
Thank you
 References

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