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JACC Case Rep
JACC Case Rep
JACC Case Reports
2666-0849
Elsevier

S2666-0849(24)00237-7
10.1016/j.jaccas.2024.102444
102444
Mini-Focus Issue on Congenital Heart Disease
Case Report: Clinical Case
Noninvasive Hemodynamic Assessment for Late-Stage Truncus Arteriosus Management
Lehtiranta Saara MD, PhD saara.lehtiranta@pohde.fi
ab∗
Martelius Laura MD, PhD a
Ylinen Mari MD, PhD a
Ojala Tiina MD, PhD a
a Department of Pediatric Cardiology, New Children Hospital Helsinki, Helsinki, Finland
b Department of Pediatrics and Adolescent Medicine, Oulu University Hospital, Oulu, Finland
∗ Address for correspondence: Dr Saara Lehtiranta Kasarmintie, 12 B 23, 90130 Oulu, Finland. saara.lehtiranta@pohde.fi
21 8 2024
21 8 2024
21 8 2024
29 16 10244429 12 2023
6 6 2024
14 6 2024
© 2024 The Authors
2024
https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
A 7-year-old girl with unoperated truncus arteriosus arrived in Finland as a refugee. In contrast to our previous practices, hemodynamic assessment was made using cardiovascular magnetic resonance instead of cardiac catheterization. During the 2-year follow-up period, the right ventricular pressure was estimated to be 50% of systemic pressure in echocardiogram evaluations.

Graphical Abstract

Key Words

cardiovascular magnetic resonance
late repair of congenital heart defect
truncus arteriosus
Abbreviations and Acronyms

CMR cardiovascular magnetic resonance

CT computed tomography

RV right ventricular
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pmcHistory of Presentation

A 7-year-old girl arrived in Finland as a refugee from Burundi. In the country of origin, the child had been diagnosed with truncus arteriosus. The general appearance of the child was good, though she experienced fatigue during exercise and recurrent coughing. Her oxygen saturation level was 100% and hemoglobin was 148 g/L. Blood pressure was 103/49 mm Hg. The growth of the child was steady: weight 18.2 kg and height 114 cm.Learning Objectives

• To emphasize the role of CMR as a noninvasive way for the assessment of hemodynamics in complex congenital heart defects evaluated in late childhood.

• To describe the comprehensive evaluation of an immigrant child with untreated complex congenital heart defect.

Past Medical History

The child had a history of multiple hospitalizations due to upper respiratory tract infections and acute heart failure. She was on diuretic therapy but had not previously undergone any cardiac operations.

Differential Diagnosis

The presence of relevant pulmonary hypertension was ruled out with cardiovascular magnetic resonance (CMR) preoperatively.

Investigations

The diagnosis of truncus arteriosus type 2A with a right aortic arch and aberrant left subclavian artery was confirmed through an echocardiogram examination and computer tomography (CT) imaging (Figure 1). A moderate truncal valve leaked with holodiastolic flow reversal in the descending aorta was also seen on echocardiography. To evaluate the appropriateness of surgical intervention, a hemodynamic assessment was conducted with CMR without the need for anesthesia.Figure 1 Preoperative Computed Tomography

Preoperative computed tomography of truncus arteriosus type 2A with right aortic arch and aberrant left subclavian artery.

The CMR results revealed enlarged ventricles with normal left ventricular function and slightly reduced right ventricular (RV) function. Additionally, a mild-to-moderate truncus valve leak (19%) was observed. The perfusion rates for the right and left pulmonary arteries were 37% and 63%, respectively. The calculated QP/SP ratio was 3.5 (Figure 2). The CMR confirmed a significant shunt to the pulmonary arteries, while the child maintained oxygen saturation within the normal range.Figure 2 Blood Flow Assessed Preoperatively With Cardiovascular Magnetic Resonance

Hemodynamic assessment with cardiac magnetic resonance. The values are expressed in mL/min/m2. The Qp/Qs ratio is calculated as the sum of the right pulmonary artery (RPA) and left pulmonary artery (LPA) divided by the sum of the descending aorta (AO desc) and superior vena cava (SVC), resulting in a ratio of 3.5. AO asc = ascending aorta.

Based on the assessment, the pulmonary pressure was determined to be sufficiently low, eliminating the need for prior hemodynamic assessment using intravenous catheter measurements. As a result, it was deemed safe to proceed with surgical repair of the truncus arteriosus.

Management

The child had endured life in a refugee camp, where she lacked access to proper medical care and nutrition. In addition to being diagnosed with congenital heart disease, the child also experienced a chronic lung infection, which was detected through a CT scan, along with protein malnutrition and dental caries. To enhance the child’s overall well-being prior to surgery, multiprofessional assessment and care was needed.

The surgical repair with the RV outflow tract reconstruction with a homograft of 20 mm, closure of ventricular septal defect, and plastic to the 4-leafed truncal valve was successfully conducted without encountering any major complications (Figure 3). The cross-clamp time was 3 hours and 34 minutes and the bypass time was 5 hours and 13 minutes.Figure 3 Postoperative Computed Tomography

Postoperative computed tomography of truncus arteriosus type 2A with right aortic arch and aberrant left subclavian artery after late surgical repair at 7 years of age.

During the recovery period, the child faced significant problems. On the first day following the operation, the child experienced an episode of ventricular tachycardia, necessitating resuscitation on 2 occasions. Rhythm disturbances persisted throughout the initial 7 postoperative days, requiring the administration of arrhythmic medication. Weaning the child off the respirator proved to be difficult due to recurrent mucus secretion and lung infection, ultimately leading to the decision to perform a tracheostomy on the 15th day after the operation. After a recovery period of 1.5 months, successful decannulation was achieved. Although inhaled nitric oxygen was used postoperatively, in repeated echocardiogram evaluations, RV pressure was estimated to be 50% of systemic pressure, so sildenafil medication was started.

Discussion

In this case, we present a refugee child who required a delayed operation for truncus arteriosus type 2. It is widely recognized that late repair (>3 months of age) of truncus arteriosus is associated with an increased risk of pulmonary hypertension crises, heart failure, and even death.1 There is only limited evidence regarding the safety of late repair for truncus arteriosus; however, the suggestion is that late surgery is possible if there are signs of a persistent left-to-right shunt accompanied by high oxygen saturation levels (up to 88%).

In our patient, we demonstrated a significant left-to-right shunt using CMR imaging with a Qp/Qs ratio of 3.5. Based on this finding, we proceeded with surgery without further invasive hemodynamic examinations. Though we optimized nutrition and treated the lung infection prior to the repair surgery, the recovery was complicated by recurrent lung infections. Effective collaboration between different specialties is crucial to ensure the best possible surgical outcomes for refugee children with severe congenital heart defects.

While refugee children with severe congenital heart defects face significant challenges in accessing timely diagnosis and treatment, advances in imaging techniques offer hope for evaluating the feasibility of surgical interventions, even in cases of late diagnosis or treatment. By fostering effective collaboration between specialties and addressing existing gaps in health care access, we can strive to improve the surgical outcomes and overall well-being of refugee children affected by congenital heart diseases.

Follow-Up

The follow-up examinations revealed an increase in truncus valve leakage (45%), necessitating a subsequent operation to implant a mechanical valve after a 1.5-year follow-up. Fortunately, the child experienced a relatively short recovery period following this procedure. In the latest echocardiogram evaluation, RV pressure estimated by echocardiography was 50% of systemic pressure (Figure 4). Cardiac catheterization was made 1.5 years postoperatively and mild pulmonary hypertension (mean pulmonary artery pressure of 20 to 25 mm Hg) was diagnosed. Therefore, the patient is still on sildenafil medication.Figure 4 Tricuspid Valve Leakage in the Latest Echocardiogram

The latest echocardiogram evaluation of right ventricular pressure estimated from tricuspid valve leakage after late surgical repair of truncus arteriosus. The examination was performed 1 year after the operation. The systemic blood pressure was 100 to 110 mm Hg while examination.

Conclusions

We demonstrated a successful outcome through late surgical repair for truncus arteriosus in a case involving a significant preoperative CMR-measured left-to-right shunt. These findings emphasize the importance of providing specialized care and tailored interventions to address the distinct health care needs of refugee children and thus achieve positive health outcomes.

Funding Support and Author Disclosures

The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the Author Center.
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Reference

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