A rare case of hemitruncus arteriosus in congenital heart disease: a clinical case report

¹Department of Medical Surgical Nursing, Smt. Radhikabai Meghe Memorial College of Nursing, Datta Meghe Institute of Higher Education and Research, Wardha, Maharashtra, India

^&Corresponding author
Tarun Ajit, Department of Medical Surgical Nursing, Smt. Radhikabai Meghe Memorial College of Nursing, Datta Meghe Institute of Higher Education and Research, Wardha, Maharashtra, India

Introduction    

The decision was based on the idea of congenital heart disease (CHD), which encompasses a large variety of structural defects of the heart and great vessels [1]. One of the least common among them is hemitruncus arteriosus, which is less than 0.1 to 100 percent of all cases of CHD [2]. It is typified by the abnormality of one pulmonary artery - usually on the right via the ascending aorta, and the other pulmonary artery has a normal origin of the pulmonary trunk [3]. This structural abnormality exposes one lung to systemic blood pressure, causes the pulmonary vessels to become congested, increases the resistance of the pulmonary veins, overloads the right ventricles, and eventually results in systemic heart failure unless surgical repair is undertaken [4].

In the absence of early surgical repair, the prognosis is dismal, with the majority of the affected infants dying before the first year of age [1]. Non-invasive diagnostic imaging has, however, made significant improvements in early diagnosis, especially through echocardiography and CT angiography, which have enabled timely surgical treatment, and this has significantly enhanced survival rates [5]. The current report describes a unique case of hemitruncus arteriosus in a neonate and highlights the need to recognize this condition and provide complex treatment along with surgery to attain a good prognosis [4].

Patient and observation     

Patient information: the case presents as a one-year-old male who suffered an uneventful pregnancy and delivery but was admitted to the hospital with complaints of worsening respiratory distress, recurrent chest infections, cyanosis, poor feeding, and lack of weight gain since the age of three months. The family history did not report any history of consanguinity or known genetic or congenital heart disease. The milestones of development in the child were slowed down, which was mainly because of chronic illness and inappropriate nutrition.

Clinical findings: the infant was observed to be very ill, undernourished, and tachypneic on a general examination.

Vital parameters: the patient was found to have a heart rate of 136 beats per minute, respiratory rate of 62 breaths per minute, and blood pressure of 90/55 mmHg. The level of oxygen saturation of the room air was registered at 76 percent. The patient was centrally cyanotic, and he had clear chest retractions and poor peripheral perfusion. Examination: cardiovascular examination disclosed a loud systolic murmur which was best heard at the left upper sternal border with palpation of the right ventricular heave as well as hepatomegaly, which is a typical characteristic of right-sided heart failure. Lung auscultation was positive, and there were fine crepitations in both lungs, indicating lung congestion.

Timeline of current episode: the patient had a normal pregnancy and post-partum period until she was subjected to a normal delivery at full term. He started getting frequent respiratory infections at the age of three months accompanied by cyanosis. His health status had degraded half a year later because he was seemingly not prospering and had breathing difficulties. Hemitruncus arteriosus was also detected at the 12-month mark as indicated by diagnostic tests. Sometime after the operation, the child also exhibited a fast clinical recovery, including a significant increase in feeding tolerance, an increase in oxygen saturation, and a visible weight gain.

Diagnostic assessment: initial evaluation began with a chest X-ray, which revealed cardiomegaly along with prominent pulmonary vascular markings, suggestive of increased pulmonary blood flow (Figure 1).

A transthoracic echocardiogram was subsequently performed, which demonstrated severe pulmonary hypertension and raised suspicion of an anomalous origin of the right pulmonary artery. The echocardiographic windows suggested abnormal flow patterns and discontinuity between the main pulmonary artery and the right pulmonary artery (Figure 2). To confirm the diagnosis, a CT angiography of the heart and great vessels was obtained. The imaging clearly delineated the right pulmonary artery arising directly from the ascending aorta, confirming the diagnosis of hemitruncus arteriosus. No other major structural cardiac abnormalities were identified (Figure 3).

Baseline laboratory investigations, including complete blood counts and metabolic profile, were within normal limits except for mild respiratory acidosis on arterial blood gas analysis, consistent with the infant's tachypnea. These multimodal imaging findings established the diagnosis and guided the decision for urgent surgical correction.

Diagnosis

Final diagnosis: right-sided hemitruncus arteriosus with secondary pulmonary hypertension.

Therapeutic interventions: the patient had clear-cut surgery with cardiopulmonary bypass. The surgery involved the dislodgement of the right pulmonary artery at the aberrant origin at the ascending aorta and reconnection into the main pulmonary artery with an end-to-end anastomosis (Figure 4). There were no complications during the intraoperative period.

The management used postoperative: the patient had successful extubation 48 hours post-operatively and maintained an oxygen saturation of approximately 96% on room air. Intravenous diuretics and antibiotics were given to ensure postoperative care, along with nutritional support for the acceleration of healing and recovery. Close cardiac and respiratory monitoring was conducted in the intensive care unit. The surgical intervention was effective in that pulmonary circulation had been normalized and strain on the right side of the heart was minimized.

Follow-up and outcomes: after the surgery, the child demonstrated some significant improvement in respiratory function, feeding skills, and the general level of activity. Follow-up: echocardiographic analysis established a patent anastomosis, and pressures in the right pulmonary artery returned to normal (Figure 5). The patient was discharged on the twelfth postoperative day in a stable state with good oxygen levels and weight gain. One month later, the child recorded normal growth parameters and developmental advancements, with no more respiratory distress or cyanosis.

Patient perspective: parents of the child were extremely thankful for the positive success of the surgery. They claimed they saw a great deal of improvement in the breathing, appetite, and energy levels of their child. The commitment and effective communication of the healthcare team in the diagnostic and postoperative periods were also recognized by the family.

Informed consent statement: written informed consent was obtained from the patient for publication of this case and accompanying images.

Discussion     

Hemitruncus arteriosus is a very uncommon and potentially fatal congenital cardiovascular malformation. It is also referred to as the abnormal origin of one pulmonary artery from the ascending aorta. In most documented cases, the left pulmonary artery originates normally from the main pulmonary trunk, whereas the right pulmonary artery arises abnormally from the ascending aorta [1,6]. The affected lung is exposed to systemic arterial pressures due to this abnormal vascular configuration, which causes vascular remodeling, excessive pulmonary blood flow, and early-onset pulmonary hypertension [7].

Symptoms that are commonly seen include tachypnea, cyanosis, frequent chest infections, poor feeding, and growth failure and are usually manifested in infants during the first few months of life. The mortality rate of the condition is high, with over 70 percent of untreated cases reported to die at the first year of life because of progressive right ventricular failure and irreversible pulmonary vascular disease. Hence, there is a need to recognize the condition at an early stage and quickly perform surgery to enhance prognosis [8]. Echocardiography is the major method of diagnosis as it can show the unusual origin of one of the pulmonary arteries. Cardiac catheterization and CT angiography can be used to add more anatomical and hemodynamic data to prove the diagnosis and guide the surgeon [9].

Standard therapy is surgery to reimplant the abnormal pulmonary artery into the great pulmonary artery, and thus reinstate normal bifurcation, and also limit the exposure of the systemic blood to the diseased lung. It is a process that, ideally, should be done in infancy; it helps to avoid the irreversible pulmonary vascular changes and significantly improves the survival rates [4,10].

Early diagnosis and subsequent timely surgical repair resulted in a great postoperative outcome in this case. The speed of oxygenation and growth of the child and his overall state of affairs demonstrate the principle of multidisciplinary cooperation: pediatric cardiologists, cardiac surgeons, anesthesiologists, and special nursing personnel should be involved as the key elements in achieving the best results.

The case underlines the fact that hemitruncus arteriosus, despite being a rare condition, is always a possibility in infants who present with unexplained respiratory distress, cyanosis, and cardiac overload of the right side. Early imaging and intervention have continued to be the foundations of the management, and they provide an opportunity for full recovery and normal growth [7].

Conclusion     

The hemitruncus arteriosus is a rare but surgically fixable congenital cardiac defect, which may cause severe cardiopulmonary complications when untreated. The development of pulmonary hypertension and cardiac failure should be avoided, and it is necessary to diagnose it early with sophisticated imaging methods and provide prompt treatment with surgery. The case presents the importance of timely diagnosis, successful surgical repair, and multidisciplinary care in enhancing the survival rates and the long-term outcomes of children with this rare disease.

Competing interests     

The authors declare no competing interests.

Authors' contributions     

Tarun Ajit: data collection and manuscript preparation were carried out by the patient; Aarti Raut: Conceptualization, surgical data analysis, and review of the manuscript; Ruchira Ankar: literature search and interpretation of the data; Samruddhi Gujar: manuscript editing and language correction; Archana Dhegare: approval of references and final manuscript. All the authors read and approved the final version of this manuscript.

Acknowledgements     

The authors wish to thank the Pediatric Cardiology and Cardiothoracic Surgery Departments of Acharya Vinoba Bhave Rural Hospital, Wardha, for their priceless experience and clinical assistance. The nursing staff is also commended to do an excellent postoperative care and their kind treatment of the patient during recovery, which is also worthy of special thanks.

Figures     

Figure 1: chest X-ray showing cardiomegaly with prominent pulmonary vascular markings (yellow arrow: right upper lung field; green arrow: left upper lung field; blue arrow: right lower lung field; red arrow: left lower lung field; black double-headed arrow: transverse measurement indicated in the radiograph; R: right side of the patient)

Figure 2: echocardiography image demonstrating suspected anomalous origin of the right pulmonary artery (AO: aorta; MPA: main pulmonary artery; RPA: right pulmonary artery; LPA: left pulmonary artery; the horizontal yellow arrow (⃗) indicates the right pulmonary artery (RPA), and the vertical yellow arrow (↑) indicates the left pulmonary artery (LPA))

Figure 3: computed tomography angiography clearly showing anomalous origin of the right pulmonary artery arising from the ascending aorta (RPA: right pulmonary arteries; LPA: left pulmonary arteries; MPA: main pulmonary artery; Ao: aorta)

Figure 4: intraoperative view identifying the right pulmonary artery originating from the ascending aorta before reimplantation (Asc Ao: ascending aorta; MPA: main pulmonary artery; RPA: right pulmonary artery; the labels indicate the corresponding anatomical structures identified during the surgical procedure)

Figure 5: postoperative echocardiography demonstrating good flow through the reimplanted right pulmonary artery with reduced pulmonary artery pressure (AAo: ascending aorta; RPA: right pulmonary artery; A: two-dimensional echocardiographic image; B: color Doppler echocardiographic image)

References