Authors: Redmond Burke, MD, Cardiovascular Surgery Chief and Anthony Rossi, MD, Chief, Cardiovascular Medicine.
The Heart Program at Nicklaus Children’s Hospital remains dedicated to providing the very best and safest experience for patients undergoing congenital heart surgery. This report will review the program’s 24-year surgical experience in repairing children with a diagnosis of truncus arteriosus.
We reviewed our CardioAccess database for patients undergoing repair of truncus arteriosus from September 1995 through February 2019.
Operative digital imaging of each patient’s procedure was routinely used to document the anatomy and repair of each lesion. Every operation was videotaped with a camera at the head of the operating table, the video was visible to the entire team during the procedure, and was digitally stored for on-demand performance review.
Clinical Surgical Pearls, Redmond Burke 2019:
Cannulation: Bicaval cannulation, clamp and cardioplegia, left atrial vent.
For Truncus with IAA: Cool to 20C, go to low flow bypass with 10 Fr. cannula in innominate artery, mobilize descending aorta with cautery, take down to third intercostal branch as needed. Pull aorta up and anastomose to ascending aorta at innominate level. De-air and reposition aortic cannula, resume full flow and rewarm to 28 degrees.
For Truncus Repair: High cross clamp, make small transverse incision in anterior ascending aorta, check position of coronary ostia, then transect aorta leaving the two pulmonary arteries together as a button, if possible. Inspect truncal valve, and repair if needed. Fill in pulmonary artery button site with native pericardial patch using running 7-0 Prolene, recheck coronary ostia. Reconnect ascending and descending aorta with running 7-0 Prolene, Make RV incision avoiding coronary branches, then resect muscle bundles in RVOT. Trim native pericardial patch to shape of VSD. Repair VSD with no touch technique using running 8-0 Prolene.
Prepare pulmonary homograft (size based on patient and availability – 7 to 12mm), trim to correct length, distal anastomosis to pulmonary artery button with 7-0 Prolene. Proximal homograft cuff sutured to distal RV incision epicardium. Create pericardial hood for the anterior cuff of the homograft and the remainder of the RV incision, running 7-0 or 6-0 Prolene. Open right atrium and repair ASD with 7-0 Prolene, close atrium with 7-9 Prolene in two layers.
Rewarm, intraoperative TEE, check arch gradient if IAA, open chest selectively, pacing wires only if needed (rare).
There were 41 neonates and Infants who underwent truncal repair during this time period (21 male, 20 female). One preterm, low-birthweight neonate with truncus arteriosus and an interrupted aortic arch patient had been palliated with bilateral pulmonary artery bands prior to repair. Complete repair was performed 26 days after the initial palliation. There were 5 patients who required repair of interrupted aortic arch and 1 patient underwent a truncal valve repair at the time of surgery. The median weight at the time of surgery for these children was 3 kgs (range 1.5-4.3 kg). There were 21 patients <3 kg and 9 <2.5 kg at the time of surgery. They were operated on at a median age of 10 days (2-85 d). There were 34 neonatal operations. There was 1 death (98% survival rate). The median postoperative stay was 17 days.There were 11 patients with a documented chromosomal anomaly or phenotypic syndromic. Cardiopulmonary bypass times averaged 183 minutes.Aortic cross clamp time was 107 minutes. Three patients required hypothermic circulatory arrest of greater than 10 minutes.
There were no patients who experienced postoperative heart block and required a permanent pacemaker. There are 20 patients who have undergone subsequent surgical palliations. Time from initial truncal repair to next surgery was at a median of 962 days (9-5614 d). Right ventricular outflow reconstruction with insertion of a right ventricle to pulmonary artery conduit was performed in 21 patients. There were 3 patients who had pulmonary artery reconstruction and 1 who required an aortic arch repair.
All patients were discharged home in good condition.
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|ASO and ASO/VSD
In 1949, Collett and Edwards published their anatomic calcification of patients with persistent truncus arteriosus, a classification which has withstood the test of time1. According to their report, “a persistent truncus arteriosus consists of a single arterial trunk arising from the ventricular part of the heart.”Also, “the single trunk receives blood from both ventricles and supplies the coronary, pulmonary and systemic circulations.”
In 1968, McGoon first described the physiologic correction of this lesion. He believed insertion of a homograft from the right ventricle to pulmonary artery was responsible for the ultimate success of this repair2.
In their landmark report, Ebert et al. described a series of patients who underwent this procedure in the first six months of life3. They also noted the importance of using a valve conduit and operating at an age before the development of pulmonary vascular disease. In this series, 89 of 100 infants operated on survived to discharge.
In the early 1990s Hanley et al in Boston began to champion the concept of neonatal truncus repair4.
They noted that postoperative complications such as pulmonary hypertension were less common when truncus was repaired in the neonatal period. Since that report, a number of centers have shown neonatal repair of truncus can be done safely and with few complications 5,6.
Today, neonatal repair of truncus in more commonplace. In a review of nearly 600 patients by the STS in 2012, mortality for the entire group was about 11%. Those patients who required repair of an interrupted aortic arch or truncal valve repairs were at highest risk. Of the 5 patients who required both of these procedures only one ultimately survived7.
Our program has had a strategy of early repair of truncus arteriosus, with over 80% of babies undergoing repair in the first month of life. Philosophically, we believe patients diagnosed with truncus arteriosus benefit from early surgery and avoidance of their disadvantaged preoperative physiologic state which can only worsen after birth, as the pulmonary vascular resistance begins to drop and the potential for a steal of coronary and systemic blood flow is real. Typically, patients admitted to our institution are scheduled for surgery in the first week of life. This includes preterm and low birth weight neonates. These babies inherent unfavorable physiologic state means waiting for somatic growth is likely to result in poor weight gain and worsening heart failure prior to surgery.
Take Home Message:
In the current era, complete repair of patients with truncus arteriosus can be accomplished in the neonatal period in most patients with low mortality and a low incidence of long-term morbidity. Reconstruction of the RVOT is a common subsequent reoperation and often involves placement or replacement of a conduit from the right ventricle to the pulmonary artery. Patients presenting in extremis or with other significant comorbidities may be palliated for short periods of time while recovering and awaiting complete repair. Meticulous surgical technique can reduce the incidence of most postoperative morbidity including the development of tachyarrhythmias or heart block.
Very dysplastic truncal valve. Image courtesy Dr. Redmond Burke.
Outcomes for cardiac surgical patients at Nicklaus Children’s Hospital continued to be reported in real-time and have been so since 2001. Nicklaus Children’s Hospital’s Real-Time Outcomes is the most comprehensive and transparent outcomes reporting website available today, with over 18 years of outcomes available for review.
- Collett RW, Edwards JE. Persistent truncus arteriosus: a classification according to anatomic types. Surgical Clinics of North America. 1949 Aug 1;29(4):1245-70.
- McGoonDC,RastelleGC,OngleyPA. An operation for the correction of truncus arteriosus. JAMA1968;205:69.
- Ebert PA, Turley KE, Stanger PA, Hoffman JI, Heymann MA, Rudolph AM. Surgical treatment of truncus arteriosus in the first 6 months of life. Annals of surgery. 1984 Oct;200(4):451.
- Hanley FL, Heinemann MK, Jonas RA, Mayer JJ, Cook NR, Wessel DL, Castaneda AR. Repair of truncus arteriosus in the neonate. The Journal of thoracic and cardiovascular surgery. 1993 Jun;105(6):1047-56.
- Kalavrouziotis G, Purohit M, Ciotti G, Corno AF, Pozzi M. Truncus arteriosus communis: early and midterm results of early primary repair. The Annals of thoracic surgery. 2006 Dec 1;82(6):2200-6.
- Bove EL, Lupinetti FM, Pridjian AK, Callow LB, Snider AR, Rosenthal A. Results of a policy of primary repair of truncus arteriosus in the neonate. The Journal of thoracic and cardiovascular surgery. 1993 Jun;105(6):1057-65.
- Russell HM, Pasquali SK, Jacobs JP, Jacobs ML, O'brien SM, Mavroudis C, Backer CL. Outcomes of repair of common arterial trunk with truncal valve surgery: a review of the society of thoracic surgeons congenital heart surgery database. The Annals of thoracic surgery. 2012 Jan 1;93(1):164-9.
Additional References of Interest:
- Anderson RC, Obata W, Lillehei CW. Truncus arteriosus: Clinical study of fourteen cases. Circulation. 1957 Oct;16(4):586-98.