Glenn Procedure in a Baby with Single Pulmonary Artery and Single Lung

Abstract

Glenn followed by Fontan are still the main two procedures for the treatment of patients with single ventricle. Some patients may be challenging due to unfavorable cardiopulmonary anatomy. In this report, we present Glenn shunt procedure in a patient with single pulmonary artery and congenitally agenetic lung.

INTRODUCTION

Single ventricular heart defects can rarely be converted to biventricular anatomy in the current era despite advances in medicine. Glenn shunt followed by Fontan procedure is still the logistic treatment or rather palliation options for the patients with single ventricle cardiac pathology.

In addition to the complexity of single ventricle, in some patients, extracardiac anatomy may also be complicated and challenge the surgical interventions. In this report, we present Glenn shunt procedure in a baby with single lung.

CASE PRESENTATION

The patient was a 9-month-old female baby without in utero diagnosis and follow-up. She was bought to the clinic with the complaint of cyanosis. She was 6600 g and had normal growth according to her age. Her room air oxygen saturation was 64%. Physical examination revealed absent pulmonary sounds on left chest and cyanosis, otherwise normal. Chest X-ray indicated absent pulmonary tissue on the left side of the thoracic cavity, hyperinflated right lung, and leftward deviated heart [Figure 1]. Echocardiography showed tricuspid atresia, double outlet right ventricle, single ventricle physiology, pulmonary artery stenosis, large secundum atrial septal defect, noncommitted ventricular septal defect, 15 mm diameter mitral annulus, 65 mmHg pulmonary gradient, and right arcus aorta.

Figure 1.

Chest X-ray indicated absent pulmonary tissue on the left side of the thoracic cavity and leftward deviated heart

After the consent of the patient's family, Glenn shunt procedure was planned. Following median sternotomy, pericardium was opened, and the left pulmonary artery and the left lung agenesis were detected and the diagnosis was confirmed [Figure 2]. Following 300 IU/kg systemic heparin injection, aortic, high superior vena caval, and inferior vena caval cannulations were performed. Azygos vein was ligated. Superior vena cava was divided from the right atrium, and the Glenn procedure was completed with anastomosis of the superior vena cava to the superior of the left pulmonary artery [Figure 3, Video 1]. The operation was finished uneventfully with oxygen saturation of 99% at 50% fraction of inspired oxygen content.

Figure 2.

The perioperative image showing the absence of left pulmonary artery and lung

Figure 3.

The peroperative image showing superior vena cava-pulmonary artery anastomosis and absence of the left pulmonary artery

The patient was taken to the Intensive Care Unit and extubated in 6 h. She was taken to the ward on the 3^rd postoperative day. The follow-up period was uneventful; however, required chest physiotherapy and the room air oxygen saturation ranged between 88% and 94%. The control echocardiogram revealed successful procedure, and she was discharged from the hospital on the postoperative 8^th day.

DISCUSSION

The incidence of congenital heart diseases is approximately 2.5–3/1000 live births, and the treatment modalities may include palliative procedures.[1] Glenn shunt procedure was first performed in 1958 by Dr. William Glenn, and it is still a useful stage in palliation for patients with single ventricle cardiac pathology.[2]

In single ventricle congenital heart defects, a staged approach is the standard surgical therapy. Due to the increased pulmonary vascular resistance, the palliative procedures are necessary to prepare patient for the Fontan procedure and to provide survival. The Glenn shunt is one of the most important stages of this pathway to the Fontan procedure.[3] It decreases pulmonary vascular resistance and relieves ventricular volume overload, hence, allows long-term survival and stage palliation in single ventricle heart defects, one-and-a-half ventricle physiology, and provides a bridge to two-ventricle repair in selected cases.[2]

In some patients, anatomic variations may be challenging for the procedure. The single ventricle cardiac heart disease together with single lung may cause a serious event resulting increased pulmonary artery resistance.[4] However, the single lung is not considered as a contraindication for the operation; the pulmonary vascular situation is one of the most important factors for the best outcome and the operation deserves extra attention.[5]

A review of the literature reveals rare cases that underwent Fontan procedure with single lung. Mostly, it is shown that the main difference between patients with confluent pulmonary arteries and patients with single lung was the postoperative arterial oxygen saturation that was lower in one-lung patients.[6] In selected patients, even if the presence of the single lung, the Fontan procedure was performed successfully with a good outcome.[4] Some long-term experiences showed that Glenn shunt to one lung may cause some late problems such as arteriovenous malformations and decreasing pulmonary functional capacity.[7] Furthermore, pulmonary vasoconstriction depending on hypoxia, hypercarbia, and increased airway pressure may result with high pulmonary vascular resistance. This makes one-lung ventilation one of the most important events and nitroglycerin, inhaled nitric oxide, and intravenous milrinone may be used in management to decrease pulmonary vascular resistance.[8]

Our patient had single pulmonary artery and lung. We decided to perform Glenn shunt as a stage to the Fontan procedure after detailed examination of the case and consent of the family The procedure was simple and uncomplicated; however, the patient required a little more chest physiotherapy attention in the postoperative period to keep arterial oxygen saturation as high as 94%.

CONCLUSION

Even though more experience is necessary for the long-term results, in the selected one-lung patients, the Glenn procedure may be performed with careful planning and result with a good outcome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.