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 Table of Contents  
Year : 2019  |  Volume : 3  |  Issue : 3  |  Page : 177-179

Neonate in Respiratory Distress with To-and-Fro Shunt from Aorta to Right Ventricle

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

Date of Submission24-Oct-2018
Date of Decision30-Nov-2018
Date of Acceptance08-Jan-2019
Date of Web Publication18-Dec-2019

Correspondence Address:
S. G. Shyam Lakshman
Department of Cardiology, Government Medical College, Kozhikode - 673 008, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiae.jiae_42_18

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A cyanotic newborn in heart failure on diagnostic transthoracic echocardiography revealed pulmonary atresia with intact ventricular septum with a vertical patent ductus arteriosus supplying from the underside of arch of aorta. There was an anomalous origin of right coronary artery from the left coronary artery. It was also associated with a coronary cameral fistula with a continuous to-and-fro shunt from the aorta through the dilated coronary to the right ventricle with a bidirectional shunt. We intend to report this case because because pulmonary atresia with single coronary artery with coronary cameral fistula is uncommon and are sparsely reported, and sparsely reported, and and are best managed by univenventricular repair followed by cardiac transplanatation.

Keywords: Anomalous coronaries, pulmonary atresia with an intact ventricular septum, to-and-fro shunt

How to cite this article:
Lakshman SG, Rajesh GN, Sajeev CG. Neonate in Respiratory Distress with To-and-Fro Shunt from Aorta to Right Ventricle. J Indian Acad Echocardiogr Cardiovasc Imaging 2019;3:177-9

How to cite this URL:
Lakshman SG, Rajesh GN, Sajeev CG. Neonate in Respiratory Distress with To-and-Fro Shunt from Aorta to Right Ventricle. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2019 [cited 2020 Aug 14];3:177-9. Available from: http://www.jiaecho.org/text.asp?2019/3/3/177/273298

  Introduction Top

Pulmonary atresia with intact ventricular septum is associated with ventriculoarterial connections and coronary cameral fistula. Transthoracic echocardiography (TTE) can be used as a simple and effective screening tool for the assessment of morphology of the right ventricle (RV) and competency of tricuspid valve, which adds to hemodynamic and angiographic data for stratification of patients for biventricular, one and a half, univentricular, or Fontan repair. In patients with pulmonary atresia with RV–coronary fistulous connections, a combined approach involving the single-ventricle strategy initially followed by heart transplantation may be the preferred strategy.

  Clinical Presentation Top

A male neonate on day 1 presented to the cardiology department with cyanosis and dyspnea. On examination, he was tachypneic, had bounding pulses, and had a left parasternal continuous murmur. Roentgenogram revealed a mild cardiomegaly with pulmonary oligemia.

The patient was subjected to two-dimensional (2D) TTE for further evaluation. 2D TTE showed normal cardiac situs with a normally related great artery relationship and a normal pulmonary venous drainage and intact atrial and ventricular septum. RV and left ventricle (LV) were not dilated. There was no tricuspid regurgitation. The short axis at pulmonary valve level showed no distinct valve with confluent pulmonary arteries, suggestive of pulmonary atresia with an intact ventricular and atrial septum and confluent pulmonary arteries [[Figure 1]a and Video 1]. Suprasternal view showed left arch and a vertical ductus supplying the pulmonary artery from under the surface of arch of aorta [Figure 1]b. Modified short axis at aortic valve level revealed dilated left main coronary and an abnormal origin of right coronary artery from left coronary artery with dilated left coronary artery [[Figure 2]a and Video 2].
Figure 1: (a) Pulmonary atresia with intact ventricular septum with confluent pulmonary arteries. (b) Vertical ductus from underside of the arch of aorta

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{Figure 1}
Figure 2: (a) Right coronary artery originating from left coronary artery. (b) Coronary cameral fistula to right ventricle

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Color flow mode showed a coronary cameral fistula with a continuous turbulent flow from aorta which could be traced to the apex and drained into the RV [[Figure 2]b and Video 3]. Continuous-wave Doppler across the shunt revealed a bidirectional shunt from aorta to RV during diastole and RV to aorta during systole [Figure 3]a, [Figure 3]b, [Figure 3]c. The patient was immediately taken up for palliative surgery. Subsequently, the patient lost follow-up and did not revert back.
Figure 3: (a) Continuous-wave Doppler showing to-and-fro shunt. (b) Shunt from right ventricle to aorta in systole. (c) Shunt from aorta to right ventricle in diastole

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  Discussion Top

Pulmonary atresia was first described by Hunter in 1784.[1] Ventriculoarterial connections were first described by Grant[2] in 1926. The incidence of single coronary artery is about 4.2% in various necropsy studies[3] and in congenital heart diseases. However, it is unclear in the setting of pulmonary atresia with intact ventricular septum. The intramyocardial sinusoids serve as a conduit to egress blood from RV through the abnormal coronaries. Ogden[4] published a similar case report of atretic semilunar valve with competent atrioventricular valve with abnormal connections with bidirectional shunting.

In the study by Davlgnon et al.,[5] which was a clinicopathologic study of 20 hearts on necropsy, ventriculocoronary connections were present in those hearts which had a small RV cavity and competent tricuspid valve. The RV cavity itself because of its hypertrophy would offer a great resistance to ventricular filling and forward blood flow. If the tricuspid valve is competent, high intraventricular pressure develops and forces blood from the ventricle into the embryonic myocardial vessels that communicate with the developing coronary arteries. Because of the absence of flow through the atretic pulmonary valve, pressure in the proximal pulmonary artery would be lower than the pressure in the adjacent coronary system, and the fistula would develop as a source of additional flow into the pulmonary artery and serve as accessory ductus arteriosus.

Usually, flow in these fistulas is bidirectional. In the study by Sissman and Abrams,[6] the RV pressure was 190/7 mmHg and the aortic pressure was 119/65 mmHg. Thus, this gradient would favor flow from the ventricle through the intermediary fistula to the aorta during systole. During diastole, the pressure gradient would be reversed, and consequently, flow would be from the aorta into the RV. Sissman and Abrams[6] were able to demonstrate this bidirectional flow by angiography. This was the pattern observed in our case.

Management of pulmonary atresia with intact ventricular septum is individualized and multifactorial. Management of the present case would be single-stage biventricular repair as the patient had tripartite RV, no RV dysplasia, and good tricuspid valve. Five-year survival rates after surgery in the current studies report 60%–70% after staged repair.[7] In the study by Zheng et al.,[8] on comparing the mortality rates between single-stage and multistage cohort groups, the survival rates were 93.7% and 88.2% as midterm outcomes in the one-stage surgery group and staged surgery group. In our patient, a preprocedure cardiac catheterization needs to be done to demonstrate RV-dependent coronary circulation (RDCC) and RV angiogram to assess RV morphology. The final decision of biventricular versus univentricular repair is based on echocardiographic, hemodynamic, and angiographic data.

In case of RDCC, decompressing RV may result in fatal ischemia and severe LV dysfunction. In patients with coronary anomalies in addition to RDCC, the idea is unclear whether to prefer univentricular repair or cardiac transplantation as it is difficult to precisely assess coronary anatomy in neonates. Even in RDCC and fistulous connection, postrepair does not guarantee good perfusion postrepair.[9] In addition, until the time of separation of the circulations at the Fontan operation, the RV is ejecting desaturated blood into the coronary arteries, which may place the distal myocardium at risk for ischemia, in particular, during periods of high demand. When deciding on single-ventricle strategy or heart transplantation, one must weigh these concerns against the issues of organ availability, early graft failure, rejection, and coronary graft disease after heart transplantation.

In the study by Rychik et al.,[10] which categorized patients of initial surgical repair into single ventricular, biventricular, and transplantation strategy, clinical judgment of a cardiologist or a surgeon, echocardiography, and angiography was used to take decisions regarding initial strategy. Echocardiography was used to measure tricuspid valve annulus diameter and tricuspid valve Z-score. Patients who had RV dependency, univentricular, and heart transplantation showed similar results. Even though primary disease is in RV, decompression of RV can lead to LV failure, even in the absence of proximal coronary stenosis due to dual supply of coronary arteries from RV and aorta; decompressing RV may result in coronary steal of coronary blood into RV with diminished areas of perfusion distal to connection and thus precipitating LV failure.

Rychik et al.[10] demonstrated median survival rates of 56 months in patients with RV coronary connections after Fontan surgery. Whether myocardial insufficiency will appear in due course of time is unclear. Hence, in case of pulmonary atresia with RV–coronary fistulous connections, a combined approach involving the single-ventricle strategy initially, with listing for heart transplantation at the early signs of myocardial insufficiency, may be the best approach. We need to do dobutamine stress echo or thallium studies which are sensitive in detecting progressive changes in myocardial perfusion.[11]

In our case, RV angiogram and catheterization would have been helpful for planning better management; unfortunately, patient's relatives did not give consent for the same.

  Conclusion Top

Pulmonary atresia is a heterogeneous disease with varying manifestation. TTE with Doppler studies can be a very useful screening tool in assessing pulmonary atresia with ventriculopulmonary arterial connections with coronary anomalies and may serve as modality along with catheterization and angiogram in deciding for initial surgical strategy. In candidates with RV–coronary fistulous connections, single-ventricle strategy followed by heart transplantation may be preferred.

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


Conflicts of interest

There are no conflicts of interest.

  References Top

Hunter W. Three cases of mal-conformation in the heart. Med Observations and Enquiries 6: 291, 1784 (cited by Peacock: Trans Path Society [London] 20: 75, 1869).  Back to cited text no. 1
Grant RT. An unusual anomaly of the coronary vessels in the malformed heart of a child. Heart 1926;13:273-83.  Back to cited text no. 2
Alexander RW, Griffith GC. Anomalies of the coronary arteries and their clinical significances. Circulation 1956;14:800-5.  Back to cited text no. 3
Ogden JA. Secondary coronary arterial fistulas. J Pediatr 1971;78:78-85.  Back to cited text no. 4
Davlgnon AL, Dushane JW, Kincaid OW, Swan HJ. Pulmonary atresia with intact ventricular septum. Am Heart J 1961;62:690.  Back to cited text no. 5
Sissman NJ, Abrams HL. Bidirectional shunting in a coronary artery-right ventricular fistula associated with pulmonary atresia and an intact ventricular septum. Circulation 1965;32:582-8.  Back to cited text no. 6
Ashburn DA, Blackstone EH, Wells WJ, Jonas RA, Pigula FA, Manning PB, et al. Determinants of mortality and type of repair in neonates with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg 2004;127:1000-7.  Back to cited text no. 7
Zheng J, Gao B, Zhu Z, Shi G, Xu Z, Liu J, et al. Surgical results for pulmonary atresia with intact ventricular septum: A single-centre 15-year experience and medium-term follow-up. Eur J Cardiothorac Surg 2016;50:1083-8.  Back to cited text no. 8
Gittenberger-de Groot AC, Sauer U, Bindl L, Babic R, Essed CE, Bühlmeyer K, et al. Competition of coronary arteries and ventriculo-coronary arterial communications in pulmonary atresia with intact ventricular septum. Int J Cardiol 1988;18:243-58.  Back to cited text no. 9
Rychik J, Levy H, Gaynor JW, DeCampli WM, Spray TL. Outcome after operations for pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg 1998;116:924-31.  Back to cited text no. 10
Paridon SM, Galioto FM, Vincent JA, Tomassoni TL, Sullivan NM, Bricker JT, et al. Exercise capacity and incidence of myocardial perfusion defects after Kawasaki disease in children and adolescents. J Am Coll Cardiol 1995;25:1420-4.  Back to cited text no. 11


  [Figure 1], [Figure 2], [Figure 3]


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