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Year : 2020  |  Volume : 8  |  Issue : 3  |  Page : 148-150

Transcatheter pulmonary valvuloplasty with coronary balloon for critical pulmonary atresia with intact ventricular septum in a neonate

Department of Cardiology, Government Medical College, Kozhikode, Kerala, India

Date of Submission03-Jan-2020
Date of Decision17-Jan-2020
Date of Acceptance27-Apr-2020
Date of Web Publication26-Nov-2020

Correspondence Address:
Dr. Santhosh Narayanan
Department of Cardiology, Government Medical College, Kozhikode, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/heartindia.heartindia_1_20

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Pulmonary atresia with intact ventricular septum is a rare cause of cyanosis in a newborn. The prognosis and treatment of the condition depend on the pulmonary anatomy as well as the state of the right ventricle. We present a case where the patient presented with acute desaturation, and we had to do an emergency pulmonary valvotomy, using coronary wires and balloons, as a lifesaving procedure. The saturation improved on the table, and the patient could be discharged from hospital in a stable state. This particular case shows that opening an atretic pulmonary valve is a viable option and coronary wires and balloons can be used for the purpose.

Keywords: Coronary balloon, emergency pulmonary valvotomy, intact interventricular septum, pulmonary atresia

How to cite this article:
Desabandhu V, Narayanan S, S Sajeer MK. Transcatheter pulmonary valvuloplasty with coronary balloon for critical pulmonary atresia with intact ventricular septum in a neonate. Heart India 2020;8:148-50

How to cite this URL:
Desabandhu V, Narayanan S, S Sajeer MK. Transcatheter pulmonary valvuloplasty with coronary balloon for critical pulmonary atresia with intact ventricular septum in a neonate. Heart India [serial online] 2020 [cited 2021 Jan 21];8:148-50. Available from: https://www.heartindia.net/text.asp?2020/8/3/148/301591

  Introduction Top

Pulmonary atresia with intact ventricular septum is a rare congenital cardiac defect that consists of atresia of the pulmonary valve, resulting in an absent connection between the right ventricular (RV) outflow tract and pulmonary arteries. There is no communication between both ventricles. Varying degrees of tricuspid valve, RV hypoplasia, and abnormalities in coronary circulation are seen. Because of the rarity of the condition, there is lesser known about the precise management plan in acute situation.

  Case Report Top

A 1-week-old male baby diagnosed with pulmonary atresia with intact interventricular septum and was admitted in our hospital with dyspnea and cyanosis. The patient's blood oxygen saturation (SpO2) fell to 30%; he developed deep blue cyanosis, was shifted to the cardiology department in a moribund state, and was on mechanical ventilation and dual inotrope support. Blood gas analysis revealed severe metabolic acidosis. The patient was shifted to the cardiac catheterization laboratory, and an RV angiogram showed a tripartite RV with atretic pulmonary valve [Figure 1]. No ventriculocoronary connections were noted on the RV angiogram. An initial attempt to cross the valve with a Terumo wire (Terumo Medical Corporation, Somerset, NJ, USA) failed. This was followed by a coronary CTO wire, Progress 120 (Abbott Vascular, Santa Clara, California, USA) with balloon support and was able to cross the valve. The valve was dilated with serially larger coronary balloons: 1.25 mm × 6 mm Mini Trak (Abbott Vascular, Santa Clara, California, USA) at 10 atmosphere pressure, 2.5 mm × 15 mm Quantum Maverick (Boston Scientific, Maple Grove, Minnesota, USA) at 16 atmosphere pressure, and 3.5 mm × 8 mm Pantera Leo NC (BIOTRONIK AG, Bulach, Switzerland) at 10 atmosphere pressure [Figure 2]. After dilatation, the catheter was introduced into the pulmonary artery and an angiogram showed good pulmonary artery anatomy [Figure 3]. The patient showed dramatic improvement in his general condition. The blood SpO2 improved to 94%, cyanosis decreased, and sensorium improved gradually. Predischarge echocardiogram showed an open pulmonary valve with a peak gradient of 11 mmHg.
Figure 1: Angiogram showing atretic pulmonary valve

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Figure 2: Balloon dilatation of pulmonary artery

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Figure 3: Angiogram after balloon dilatation showing flow into the pulmonary artery

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  Discussion Top

Pulmonary atresia with intact interventricular septum is a congenital cyanotic heart disease in the neonate, occurring at a rate of 0.6/10,000 live births. The pulmonary circulation in this condition is usually dependent on a patent ductus arteriosus and rarely multiple aortopulmonary collaterals.[1] Competence of the tricuspid valve, size of the RV, ventriculocoronary connections, and coronary artery abnormalities are important factors determining the prognosis in these patients.[2] Treatment modalities include surgical correction and catheter intervention, mainly balloon pulmonary valvuloplasty. Transcatheter treatment has become popular in recent years with the improvements in techniques and pre- and post-intervention intensive care. Balloon pulmonary valvuloplasty is usually reserved for patients in whom the coronary circulation is not RV dependent. The major determinants of a successful balloon valvuloplasty are pulmonary valve annulus diameter, tricuspid valve annulus diameter, indexed RV volume, and coronary artery anatomy.[3] In rare circumstances, these patients may be subjected to stenting of the ductus arteriosus as a bridge to surgery or following successful pulmonary valve perforation in patients whose RV is not able to maintain pulmonary circulation on its own.[4] The major determinants of success of a pulmonary valvotomy are the presence of an RV-dependent coronary circulation, tricuspid valve annulus diameter, pulmonary valve annulus diameter, and indexed RV volume. In our case, the patient had a tripartite RV without any obvious coronary cameral fistulae, and we decided to proceed with pulmonary valvotomy.[5] This report emphasizes that transcatheter management is a feasible, safe, and effective treatment in newborns.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given consent for images and other clinical information to be reported in the journal. The guardian understands that the names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

Ethical Approval

Approvalobtainedfrom institutionalethicsCommittee. Informedconsentobtained from Guardianofthepatient.

  References Top

Allen HD, Robert E, Janiel D, Timothy E, Frank, Celta, et al. Moss and Adam's Heart Disease in Infants, Children and Adolescents: Including the Fetus and Young Adult. Philadelphia, United States: Wolters Kluver; 2016. p. 1009.  Back to cited text no. 1
Cheung EW, Richmond ME, Turner ME, Bacha EA, Torres AJ. Pulmonary atresia/intact ventricular septum: Influence of coronary anatomy on single-ventricle outcome. Ann Thorac Surg 2014;98:1371-7.  Back to cited text no. 2
Fedderly RT, Lloyd TR, Mendelsohn AM, Beekman RH. Determinants of successful balloon valvotomy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum. JACC 1995;25:460-5.  Back to cited text no. 3
Guleserian KJ, Armsby LB, Thiagarajan RR, del Nido PJ, Mayer JE Jr. Natural history of pulmonary atresia with intact ventricular septum and right-ventricle-dependent coronary circulation managed by the single-ventricle approach. Ann Thorac Surg 2006;81:2250-7.  Back to cited text no. 4
Odemis E, Ozyilmaz I, Guzeltas A, Erek E, Haydin S, Bakır I. Transcatheter management of neonates with pulmonary atresia with intact ventricular septum: A single center experience from Turkey. Artif Organs 2013;37:E56-61.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3]


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