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CASE REPORT
Year : 2014  |  Volume : 41  |  Issue : 1  |  Page : 41-44

Neonatal balloon aortic valvotomy for critical aortic stenosis with congestive heart failure and severe left ventricular dysfunction


1 Department of Pediatric Cardiology, Dr. P. K. KLE Hospital & MRC, Belgaum, Karnataka, India
2 Department of Cardiac Anesthesia, JNMC, Belgaum, Karnataka, India
3 Department of Pediatrics, JNMC, Belgaum, Karnataka, India

Date of Web Publication7-Feb-2014

Correspondence Address:
Veeresh Fakeerappa Manvi
Department of Pediatric Cardiology, Dr. P. K. KLE Hospital & MRC, Belgaum, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-5009.126753

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  Abstract 

We present the case report of a 23 day newborn baby who presented with congestive heart failure. On two-dimensional echocardiography, it was diagnosed to have severe (critical) aortic valve stenosis with severe left ventricle (LV) dysfunction with dilated left atrium and left ventricle. Emergency percutaneous balloon aortic valvotomy was performed. Post-procedure the child showed immediate relief of aortic stenosis. Improvement in the left ventricular ejection fraction was also noted. Immediate and short term follow-up shows improvement in the clinical and Doppler echocardiography findings.

Keywords: Balloon aortic valvotomy, critical aortic stenosis, neonatal left ventricle (LV) dysfunction


How to cite this article:
Manvi VF, Pawar RS, Vagrali AT, Patil SS, Mahantshetti NS. Neonatal balloon aortic valvotomy for critical aortic stenosis with congestive heart failure and severe left ventricular dysfunction. J Sci Soc 2014;41:41-4

How to cite this URL:
Manvi VF, Pawar RS, Vagrali AT, Patil SS, Mahantshetti NS. Neonatal balloon aortic valvotomy for critical aortic stenosis with congestive heart failure and severe left ventricular dysfunction. J Sci Soc [serial online] 2014 [cited 2019 Jul 18];41:41-4. Available from: http://www.jscisociety.com/text.asp?2014/41/1/41/126753


  Introduction Top


Bicuspid aortic valve is the most common gross morphologic congenital abnormality of the heart or great arteries. [1] A bicuspid aortic valve can remain functionally normal throughout life time. However, more often than not fibrocalcific thickening and commissural fusion decreases the mobility and renders it stenotic. In severe cases of neonatal aortic stenosis, the left heart may not have the capacity to handle the entire cardiac output and hence closure of ductus arteriosus may result in circulatory collapse. Echocardiography with Doppler evaluation confirms the diagnosis. Careful assessment of left ventricular size and function is imperative. Initial management of neonatal critical aortic stenosis consists of maintaining ductal patency with prostaglandin infusion, inotropic support if left ventricular function is impaired and mechanical ventilation if necessary. Balloon aortic valvotomy remains the treatment of choice.


  Case report Top


A 23-day-old neonate was born to a primigravida mother by full term normal vaginal delivery. It was product of non-consanguineous marriage. Child was apparently asymptomatic and remained well until 15 days of life following which he developed hurried breathing, difficulty in feeding and excessive perspiration. He was admitted in a pediatric hospital where he had signs and symptoms of congestive heart failure. Chest radiography done, showed cardiomegaly and hence was referred to our hospital for cardiovascular system evaluation. Child had heart rate of 150/min, respiratory rate of 60/min and liver palpable. Grade 3/6 ejection systolic murmur was present in the aortic area. Two-dimensional echocardiography showed bicuspid aortic valve with critical (severe) valvar aortic stenosis [Figure 1]. There was a severe left ventricular systolic dysfunction with ejection fraction of 25%. Left atrium and left ventricle were dilated. Aortic valve annulus was 8 mm. Immediate neonatal intensive care unit (NICU) admission was followed by decongestive therapy and supportive neonatal care.
Figure 1: Echo images of valvar aortic stenosis in sub costal view and apical five chamber view with severe aortic stenosis and turbulent flow in ascending aorta

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Child was taken up for emergency balloon aortic valvotomy [Figure 2]. Child was administered conscious sedation by pediatric cardiac anesthetist. Left femoral artery was cannulated, 5F Terumo sheath was used. Ascending aortic systolic pressure was 72 mmHg. Using 5F multipurpose catheter and 0.035 Terumo straight tip guide wire, the aortic valve was crossed. Catheter was placed in the left ventricle. Left ventricular systolic pressure was 120 mmHg and the gradient across the aortic valve was 48 mmHg [Figure 3] and [Figure 4]. An Amplatz super stiff exchange wire (0.018″ × 260 cm) was placed in the left ventricle. A Tyshak II balloon (8 mm × 4 cm) was passed over the wire across the valve. The balloon was inflated twice until complete disappearance of waist under fluoroscopic and echocardiography guidance.
Figure 2: Balloon aortic valvuloplasty. (a) Aortic angiogram showing severe valvar aortic stenosis. (b) Multipurpose catheter in left ventricle. (c and d) Balloon is placed across the aortic valve and inflated

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Figure 3: Presssure tracing of aorta and left ventricle before procedure

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Figure 4: Pressure tracing of left ventricle

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Post-procedure, the left ventricular pressure dropped to 100 mmHg. Pull back gradient was 40 mmHg [Figure 5]. Echocardiography showed well-opened aortic valve with gradient of 20 mmHg. There was a significant improvement in the left ventricular systolic function. Ejection fraction was 55%. There was trivial aortic regurgitation. There were no local or systemic complications noted. Child was monitored in the NICU and discharged the next day with oral decongestive therapy. One month follow-up examination showed child had significant clinical improvement. Echocardiography showed well-opened aortic valve and normal left ventricular ejection fraction (60%).
Figure 5: Pull back pressure tracing across aortic valve showing mild residual aortic stenosis gradient of 16 mmHg

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It was the first case of balloon aortic valvotomy carried out in a neonate with severe valvar aortic stenosis and left ventricular dysfunction at our institute. The procedure was carried out free of cost under Balsanjeevini scheme of Govt. of Karnataka.


  Case discussion Top


The true incidence of congenital aortic valve stenosis is not precisely known. Bicuspid aortic valve is one of the most common congenital heart diseases and occurs in approximately 2% of the population. [1],[2],[3] Males are affected more frequently than females with ratio reported to be in the range of 3:1-5:1. [4],[5]

Bicuspid aortic valve results from partial or complete fusion of two of the aortic valve cusps with or without rapha at the site of fusion. Stenosis of the aortic valve is due to decrease is in the orifice size resulting from thickening and increased frigidity of the valve leaflets. The clinical spectrum of bicuspid aortic valve could vary from a normally functioning and malformed bicuspid aortic valve to severe aortic stenosis in fetal life resulting in hypoplastic left heart syndrome. [6]

Left ventricle subendocardial ischemia and infarction may occur in patients with valvular aortic stenosis and unobstructed coronary arteries. The cause of ischemia appears to be an imbalance between coronary blood flow to the hypertrophied left ventricle and myocardial oxygen demand that is increased owing to pressure overload. In more severe cases of neonatal aortic stenosis, the left heart may not have the capacity to handle the entire cardiac output and closure of ductus arteriosus may result in circulatory collapse. Closure of ductus may not occur for several days after birth. Initial management of neonatal critical aortic stenosis consists of maintaining ductal patency with prostaglandin E1 infusion. Balloon aortic valvotomy is the treatment of choice, although some advocate surgical valvotomy (open or trans ventricular). [7],[8] Techniques for balloon aortic valvotomy include retrograde approach via femoral, umbilical or carotid artery access or ante grade approach via patent foramen oval from venous access. [9]

Catheter-based therapy of neonatal aortic stenosis is associated with higher morbidity than in older children. Patient with a poor hemodynamic status have a higher risk of developing complications. In an early retrospective study, [10] a 9% mortality rate was related to the left ventricular perforation, balloon dilation of cusp resulting in severe aortic insufficiency, and sepsis secondary to prolonged catheter manipulation through the umbilical artery. Other studies described similar complication. [11] However, many of these complications are considered preventable. In a large study, [12] 4% early mortality was reported and among early survivors, a smaller left ventricle and a smaller aortic annulus were associated with decreased long-term survival. In the same study, moderate or severe aortic insufficiency developed in 15% of the patients after balloon valvotomy. A higher incidence of vascular complications remains a concern in newborn after aortic balloon valvotomy. Femoral artery access in neonates is followed by arterial injury and pulse loss in up to 30-39% of cases, [13] with thrombosis and potential growth retardation of the affected extremity. Catheter manipulation in the umbilical artery has been associated with vessel disruption. The transcarotid approach requires a cut down and suture of the arteriotomy at the end of the procedure and carries the potential risk of residual stenosis of the right carotid artery.

Aortic valve stenosis is a lifelong disease and the parents of these patients should be informed about the palliative nature of the procedure. A gradient reduction of more than 50% is achieved in around 70% of the patients. A low pre-dilation gradient and a small aortic annulus and aortic root have been identified as predictors of low success rates. Most studies show that at 5 year follow-up, around 85% of patients are alive and 60% remain free of reintervention.

 
  References Top

1.Roberts WC. The congenitally bicuspid aortic valve. A study of 85 autopsy cases. Am J Cardiol 1970;26:72-83.  Back to cited text no. 1
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2.Larson EW, Edwards WD. Risk factors for aortic dissection: A necropsy study of 161 cases. Am J Cardiol 1984;53:849-55.  Back to cited text no. 2
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3.Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002;39:1890-900.  Back to cited text no. 3
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4.Campbell M. The natural history of congenital aortic stenosis. Br Heart J 1968;30:514-26.  Back to cited text no. 4
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5.Frank S, Johnson A, Ross J Jr. Natural history of valvular aortic stenosis. Br Heart J 1973;35:41-6.  Back to cited text no. 5
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6.Allan LD, Sharland G, Tynan MJ. The natural history of the hypoplastic left heart syndrome. Int J Cardiol 1989;25:341-3.  Back to cited text no. 6
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7.Zeevi B, Keane JF, Castaneda AR, Perry SB, Lock JE. Neonatal critical valvar aortic stenosis. A comparison of surgical and balloon dilation therapy. Circulation 1989;80:831-9.  Back to cited text no. 7
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8.Gatzoulis MA, Rigby ML, Shinebourne EA, Redington AN. Contemporary results of balloon valvuloplasty and surgical valvotomy for congenital aortic stenosis. Arch Dis Child 1995;73:66-9.  Back to cited text no. 8
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9.Pass RH, Hellenbrand WE. Catheter intervention for critical aortic stenosis in the neonate. Catheter Cardiovasc Interv 2002;55:88-92.  Back to cited text no. 9
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10.Sholler GF, Keane JF, Perry SB, Sanders SP, Lock JE. Balloon dilation of congenital aortic valve stenosis. Results and influence of technical and morphological features on outcome. Circulation 1988;78:351-60.  Back to cited text no. 10
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11.Beekman RH, Rocchini AP, Andes A. Balloon valvuloplasty for critical aortic stenosis in the newborn: Influence of new catheter technology. J Am Coll Cardiol 1991;17:1172-6.  Back to cited text no. 11
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12.McElhinney DB, Lock JE, Keane JF, Moran AM, Colan SD. Left heart growth, function, and reintervention after balloon aortic valvuloplasty for neonatal aortic stenosis. Circulation 2005;111:451-8.  Back to cited text no. 12
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13.Magee AG, Nykanen D, McCrindle BW, Wax D, Freedom RM, Benson LN. Balloon dilation of severe aortic stenosis in the neonate: Comparison of anterograde and retrograde catheter approaches. J Am Coll Cardiol 1997;30:1061-6.  Back to cited text no. 13
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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