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Palliative vertical vein stenting in a critical neonate with obstructed infradiaphragmatic total anomalous pulmonary venous connection

Part of:Interventional Cardiology

Published online by Cambridge University Press:  02 February 2022

Kanupriya ChaturvediOpen the ORCID record for Kanupriya Chaturvedi[Opens in a new window],
Apurva Deshmukh,
Prashant Thakur,
Amit Lal and
Atul Prabhu
Kanupriya Chaturvedi
Affiliation:
Department of Pediatric Cardiology, Sri Sathya Sai Sanjeevani Hospital for Child Heart Care, Naya Raipur, India
Apurva Deshmukh
Affiliation:
Department of Pediatric Cardiology, Sri Sathya Sai Sanjeevani Hospital for Child Heart Care, Naya Raipur, India
Prashant Thakur
Affiliation:
Department of Pediatric Cardiology, Sri Sathya Sai Sanjeevani Hospital for Child Heart Care, Naya Raipur, India
Amit Lal
Affiliation:
Department of Anesthesia, Fortis Hospital, Bengaluru, India
Atul Prabhu*
Affiliation:
Department of Pediatric Cardiology, Sri Sathya Sai Sanjeevani Hospital for Child Heart Care, Naya Raipur, India
*
Author for correspondence: A. Prabhu, MD, FNB, Department of Pediatric Cardiology, Sri Sathya Sai Sanjeevani Hospital for Child Heart Care, Naya Raipur, Chhattisgarh 492101, India. Tel: +91-7977129346; Fax: +91-7712970325. E-mail: dratul.prabhu@srisathyasaisanjeevani.com
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Abstract

Neonatal total anomalous pulmonary venous connection has an incidence of about ∼1–2%.1 The clinical presentation of total anomalous pulmonary venous connection in the neonatal period is dependent on the presence or absence of pulmonary venous obstruction, which is usual when the veins connect to the umbilicovitelline system, as in infradiaphragmatic total anomalous pulmonary venous connection. Obstructed total anomalous pulmonary venous connection presents with severe respiratory distress, metabolic acidosis, and cyanosis and requires urgent surgical intervention. However, critically ill neonates with obstructed total anomalous pulmonary venous connection have a higher surgical morbidity and mortality, and pre-operative stabilisation can improve outcomes in them. We present a case of a septic term neonate with obstructed infradiaphragmatic total anomalous pulmonary venous connection who underwent emergency palliative stent placement for immediate relief of pulmonary venous obstruction.

Keywords

Infradiaphragmatic TAPVCvertical vein stenting

Information

Type
Brief Report
Information
Cardiology in the Young , Volume 32 , Issue 9 , September 2022, pp. 1536 - 1537
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Copyright
© The Author(s), 2022. Published by Cambridge University Press

Obstructed total anomalous pulmonary venous connection in a neonate usually presents with severe respiratory distress and metabolic acidosis, often requiring cardio-respiratory support and warrants immediate relief of the obstruction. Surgical intervention is the definite treatment of this condition; however, obstruction leading to severe pulmonary venous congestion, poor pre-operative clinical status, and low weight is associated with increased surgical mortality. Reference Husain, Maldonado and Rasch2,Reference Yong, d’Udekem and Robertson3 Relief of pulmonary venous obstruction via emergent vertical vein stenting to stabilise a critical neonate prior to surgery can decrease morbidity and mortality. Reference Al-Muhaya, Najjar and Jelly4,Reference Bratincsák, Van Alstine and Koren5 We report the successful relief of severe pulmonary venous obstruction in a neonate with infradiaphragmatic total anomalous pulmonary venous connection, whom we felt was unfit for emergent surgery.

Case report

A full-term, one-day-old infant was transferred to our institution from a peripheral health centre in view of cyanosis and suspicion of CHD. The baby weighed 2.3 kg on arrival, had severe respiratory distress, and was deeply cyanosed with an oxygen saturation of ∼55–60%. Given the critical condition on arrival, she was intubated and placed on mechanical ventilation. Arterial blood gas analysis showed severe metabolic acidosis (pH 7.2) and hypoxia (paO2 27 mmHg). Echocardiogram revealed situs solitus, levocardia, and atrioventricular-ventriculoarterial concordance. All four pulmonary veins formed a common chamber behind the left atrium and drained below the diaphragm into the inferior vena cava, via a descending vertical vein with obstruction at the level of the vertical vein-inferior vena cava junction. The vertical vein had a short intrahepatic course and opened almost directly in the inferior vena cava at an acute bend. There was a large patent ductus arteriosus shunting predominantly right to left and a patent foramen ovale with an obligatory right to left shunt. The infant had severe pulmonary hypertension with systolic dysfunction of the right ventricle. She was diagnosed as having obstructed infradiaphragmatic total anomalous pulmonary venous connection and further testing done for a pre-operative evaluation revealed low white blood cell counts with neutrophilia. She continued to remain critical and in severe metabolic acidosis and hypoxia with a saturation of ∼65–70% despite optimal mechanical ventilation, antibiotics, and other support measures. Repeat blood counts showed that the infant had started developing thrombocytopenia (80,000/cumm). Since the infant was unstable and unsuitable for cardiopulmonary bypass, we decided to take her to the catheterisation laboratory to relieve the vertical vein-inferior vena cava obstruction. Vascular access was obtained in the right femoral artery for continuous blood pressure monitoring, and in the right internal jugular vein through which a 5 Fr sheath was placed, and a 5 Fr multipurpose catheter was passed into the right atrium-right ventricle-inferior vena cava-hepatic vein. The opening of the vertical vein was probed with three to four sighting angiograms using dilute radiopaque contrast Visipaque®. The catheter was placed near the opening of a hepatic vein in the inferior vena cava over a 0.014 coronary wire guidewire across the constriction into the vertical vein. The position was confirmed with an angiogram which showed all four pulmonary veins forming a confluence and forming an inverted Christmas tree like pattern to drain through a slender vertical vein (Figs 1 and 2).

Figure 1.

All four pulmonary veins forming a confluence and an inverted Christmas tree like pattern to drain through a slender vertical vein. The flow through the vertical vein could not be demonstrated well as the 4 Fr catheter was completely obstructing it.

Figure 2.

Final stent position.

A premounted, balloon expandable Rebel Platinum Chromium Coronary Stent (4 mm × 20 mm) was passed over the wire and stationed at the vertical vein-inferior vena cava constriction and dilated resulting in almost immediate improvement in oxygen saturation to 80–85%. She continued to remain in same oxygen saturations and the stent remained in place for the rest of her hospital stay. However, due to multiple organ failure and severe sepsis despite optimisation of antibiotics, she ultimately succumbed before undergoing surgical repair.

Discussion

Infradiaphragmatic total anomalous pulmonary venous connection is the most common type of total anomalous pulmonary venous connection that is associated with obstruction, Reference Burroughs and Edwards6 which gives rise to severe pulmonary oedema and pulmonary hypertension and is often refractory to medical management. Surgical correction is the definitive intervention in these cases; however, in preoperatively critical neonates, surgery is associated with high mortality. Reference Yong, d’Udekem and Robertson3 Other factors like sepsis and lower patient weight can further worsen the surgical outcome. All the above factors can lead to a delay in surgery and can prove to be detrimental for the patient. Our patient was critically ill and unfit to undergo an emergent surgery. Sepsis and pulmonary venous congestion were both contributing to her critical state and despite optimal medical measures, she continued to deteriorate. Palliative stenting of the vertical vein improved her lungs and lead to increased oxygen saturations and improved blood gas, but she could not survive fulminant sepsis. It cannot be overemphasised that palliative stent placement can be considered in low weight, critically sick neonates with obstructed total anomalous pulmonary venous connection until they are in a more stable pre-operative condition or have gained weight. It can give time to a neonate to tide over an acute crisis and lead to better surgical outcomes.

Acknowledgements

None.

Financial support

This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.

Conflicts of interest

The authors have no conflict of interest.

References

Mehrizi, A, Hirsch, MS, Taussig, HB. Congenital heart disease in the neonatal period: autopsy study of 170 cases. J Pediatr 1964; 65: 721–726.10.1016/S0022-3476(64)80156-6CrossRefGoogle ScholarPubMed
Husain, SA, Maldonado, E, Rasch, D, et al. Total anomalous pulmonary venous connection: factors associated with mortality and recurrent pulmonary venous obstruction. Ann Thorac Surg 2012; 94: 825–831.10.1016/j.athoracsur.2012.04.026CrossRefGoogle ScholarPubMed
Yong, MS, d’Udekem, Y, Robertson, T, et al. Outcomes of surgery for simple total anomalous pulmonary venous drainage in neonates. Ann Thorac Surg 2011; 91: 1921–1927.10.1016/j.athoracsur.2010.12.069CrossRefGoogle ScholarPubMed
Al-Muhaya, MA, Najjar, AH, Jelly, AA, et al. Palliative emergency stenting of the vertical vein in obstructive supracardiac pulmonary venous drainage prior to surgical repair. J Saudi Heart Assoc 2019; 31: 64–68.10.1016/j.jsha.2018.12.001CrossRefGoogle ScholarPubMed
Bratincsák, A, Van Alstine, WG, Koren, L, et al. Elective stent implant in the obstructed vertical vein of supracardiac total anomalous pulmonary venous connection prior to operative repair. J Struct Heart Dis 2017; 3: 176–179.10.12945/j.jshd.2017.025.17CrossRefGoogle Scholar
Burroughs, JT, Edwards, JE. Total anomalous pulmonary venous connection. Am Heart J 1960; 59: 913–931.10.1016/0002-8703(60)90414-2CrossRefGoogle ScholarPubMed
Figure 0

Figure 1.All four pulmonary veins forming a confluence and an inverted Christmas tree like pattern to drain through a slender vertical vein. The flow through the vertical vein could not be demonstrated well as the 4 Fr catheter was completely obstructing it.

Figure 1

Figure 2.Final stent position.