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Absent Pulmonary Valve in a Case of Tetralogy of Fallot: An Incidental Discovery on Intraoperative Transesophageal Echocardiography

Gharde, Parag MD, DM*; Aggarwal, Neelam MD, DM; Sharma, Kamal Prakash MD, DM*; Gadhinglajkar, Shrinivas MD; Babu, Saravana MS, MD, DM; Chauhan, Sandeep MD*; Rajashekar, Palleti MS, Mch

doi: 10.1213/ANE.0000000000002317
Perioperative Echocardiography and Cardiovascular Education
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SDC

Published ahead of print July 21, 2017.

From the *Department of Cardiac Anaesthesia, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India; Department of Anaesthesiology, Sree Chitra Tirunal Institute for Medical Sciences & Technology, Trivandrum, Kerala, India; and Department of Cardiac Surgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.

Accepted for publication May 3, 2017.

Published ahead of print July 21, 2017.

Funding: None.

The authors declare no conflicts of interest.

Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal’s website.

A written informed consent was obtained from patient’s relative for publication of the case report.

Reprints will not be available from the authors.

Address correspondence to Neelam Aggarwal, MD, DM, Department of Cardiothoracic and Vascular Anaesthesia, Sree Chitra Tirunal Institute for Medical Sciences & Technology, Trivandrum 695011, Kerala, India. Address e-mail to neeluaggarwaldoc@yahoo.co.in.

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A 17-year-old male patient (body surface area 1.36 kg/m2) with history of cyanosis since birth due to tetralogy of Fallot (TOF) was scheduled for complete repair. The preoperative TTE evaluation revealed a large subaortic ventricular septal defect (VSD), right ventricular (RV) infundibular and pulmonic valve (PV) stenosis, and enlarged pulmonary artery (PA). Cardiac catheterization study showed RV systolic pressure of 102/10 mm Hg. There were no major aortopulmonary collaterals and coronary artery anomalies. After induction of anesthesia, a comprehensive transesophageal echocardiography examination was performed using three-dimensional (3D) matrix-array transducer probe (X7-2t; iE33; Philips, Bothell, WA). The hypertrophic and stenotic RV infundibulum coexisted with normal PA anatomy. The continuous wave Doppler across the pulmonic annulus recorded a systolic mean gradient of 60 mm Hg and flow reversal during entire diastole in upper-esophageal aortic arch short-axis view (Figure 1). Color flow Doppler analysis of right ventricular outflow tract (RVOT) depicted the site of systolic and diastolic flow aliasing at a Nyquist limit of 50–60 cm/s (Figure 2; Supplemental Digital Content 1, Video 1, http://links.lww.com/AA/B888). To focus on the PV in the imaging sector, the probe was turned to the left (counterclockwise) from midesophageal RV inflow-outflow as recommended by the American Society of Echocardiography guidelines.1 The multiplane angle was increased maintaining the leftward turn of the probe to yield the long-axis view of main PA with PV in the far field. Interestingly, only a thin rim of valve tissue was seen at pulmonic annulus in the modified midesophageal aortic valve (AV) long-axis view (Supplemental Digital Content 2, Video 2, http://links.lww.com/AA/B889). A single beat full volume 3D mode was activated in upper-esophageal aortic arch short-axis view and the acquired image was cropped from PA side to display an en-face view of pulmonic annulus with rudimentary dysplastic valve tissue (Figure 3A; Supplemental Digital Content 3, Video 3, http://links.lww.com/AA/B890). From the previous view, the 3D image was rotated upwards by 90° (along y-axis) and cropped from RV side to visualize the absence of PV leaflets (Figure 3B). The pulmonary annulus was found to be hypoplastic (major axis 14 mm; Z score: −2.9)2 in offline analysis of 3D image datasets in multiplanar reconstruction (Figure 4). The surgeon performed RV infundibular muscle resection and VSD closure with Dacron patch through transatrial approach. The rudimentary PV tissue was excised and replaced by a 22 mm size aortic homograft along with patch augmentation of the RVOT with autologous pericardium.

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DISCUSSION

TOF with an absent PV is a rare congenital anomaly with an incidence of 3%–6%.3 It is characterized by the presence of a large unrestrictive subaortic VSD, overriding aorta, RV hypertrophy with infundibular stenosis, rudimentary or completely absent PV, and dilated PAs. An absent PV results in severe pulmonary regurgitation (PR) and is associated with enlargement of PAs resulting in trachea-bronchial compression, tracheomalacia, and air trapping.4 The clinical presentation in TOF patients with absent PV varies over a wide spectrum depending on pathophysiology of the disease entity.3,4 Respiratory difficulties along with mild cyanosis are the most prominent clinical features in the majority of the neonates and infants. However, with severe pulmonic annular hypoplasia and RV infundibular stenosis as seen in our case, cyanosis and hypoxemia predominate the clinical scenario and patients follow a course similar to classical TOF patients.

In 4%–5% of TOF patients, patent ductus arteriosus or major aortopulmonary collaterals augment the systolic antegrade flow in proximal main PA. This is less often in TOF patients with an absent PV. In our case, the dilated PAs found on preoperative imaging prompted us to investigate the detailed morphology of PV. The leaflets of PV are thinner than the AV, so more suitable to image with transthoracic approach as compared with transesophageal approach.5 Moreover, American Society of Echocardiography guidelines do not recommend any dedicated two-dimensional (2D) cross-sectional views of PV as its plane is located superior and oblique to that of the AV, increasing the difficulty of image acquisition.1 Despite the above-mentioned limitations, the rudimentary PV could be identified by slight modification of the conventional 2D views as previously described in the literature.1,6 Evaluation with continuous wave Doppler revealed the additional feature of flow reversal in PA and color flow Doppler demonstrated aliasing of diastolic velocities below the pulmonic annulus due to severe PR, thus supporting the diagnosis of absent PV. In the presence of good quality 2D images, three-dimensional (3D) reconstruction may be a useful modality for morphological assessment of PV and precise estimation of annular dimensions. If TEE imaging of PV is inconsistent, epicardial echocardiography may be an appropriate alternative.

The RV infundibular hypertrophy and hypoplastic pulmonic annulus must have protected the RV from volume overload induced by severe PR, while the PAs were not massively dilated to cause any respiratory symptom. This protective effect would have been eliminated in the postoperative period after RV infundibular resection. The surgeon appropriately replaced the rudimentary PV with an adequate size homograft to provide a competent PV, which is highly desirable in this variant of TOF to prevent postoperative RV dysfunction.7

A detailed intraoperative TEE examination of PV, RVOT, and pulmonary arteries should be performed from several imaging windows to explore the TOF variants and associated anomalies.

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Clinician’s Key Teaching Points

By Massimiliano Meineri, MD, and Nikolaos J. Skubas, MD

  • In tetralogy of Fallot (TOF), patients present with an aorta overriding a ventricular septal defect, right ventricular (RV) hypertrophy, and pulmonic infundibular stenosis.
  • Absence of pulmonic valve (PV) is rare in TOF patients and results in PV regurgitation, dilation of the pulmonary arteries, RV overload, and compression of neighboring trachea and bronchi. Assessment of PV morphology by TEE is challenging due to its anterior position and thin leaflets. Spectral and color Doppler should be utilized to assess secondary anatomic and physiologic changes. Three-dimensional (3D) and epicardiac echocardiography should be used in case of suboptimal imaging quality.
  • In this case of a patient undergoing surgical correction of TOF, the PV leaflets could not be visualized with 2D TEE. Diastolic aliasing at the PV annulus by color Doppler (midesophageal RV inflow-outflow) and holodiastolic flow reversal in the pulmonary artery by pulsed wave Doppler (upper-esophageal aortic arch short-axis) were used to diagnose severe PV regurgitation. 3D imaging displayed rudimentary PV leaflets tissue and measured a hypoplastic PV annulus. PV replacement with a homograft was added to the surgical procedure.
  • Understanding the different TOF variants is key to perform a comprehensive TEE assessment. A hypoplastic PV annulus mitigates the consequences of PV regurgitation. A coexisting subpulmonic stenosis limited pulmonary artery flow and mimicked the typical TOF physiology, because it caused blood shunting across the large ventricular septal defect.
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DISCLOSURES

Name: Parag Gharde, MD, DM.

Contribution: This author helped conduct the study and prepare the manuscript.

Name: Neelam Aggarwal, MD, DM.

Contribution: This author helped conduct the study and write the manuscript.

Name: Kamal Prakash Sharma, MD, DM.

Contribution: This author helped write the manuscript.

Name: Shrinivas Gadhinglajkar, MD.

Contribution: This author helped prepare the manuscript.

Name: Saravana Babu MS, MD, DM.

Contribution: This author helped prepare the manuscript.

Name: Sandeep Chauhan, MD.

Contribution: This author helped prepare the manuscript.

Name: Palleti Rajashekar, MS, Mch.

Contribution: This author helped prepare the manuscript.

This manuscript was handled by: Nikolaos J. Skubas, MD, DSc, FACC, FASE.

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REFERENCES

1. Hahn RT, Abraham T, Adams MS, et al. Guidelines for performing a comprehensive transesophageal echocardiographic examination: recommendations from the American Society of Echocardiography and the Society of Cardiovascular Anesthesiologists. J Am Soc Echocardiogr. 2013;26:921–964.
2. Vyas H, Eidem BW. Eidem BW, Cetta F, O’Leary PW. Tetralogy of Fallot. In: Echocardiography in Pediatric and Adult Congenital Heart Disease. 2012:Philadelphia, PA: Lippincott Williams & Wilkins, 240.
3. Kirshbom PM, Kogon BE. Tetralogy of Fallot with absent pulmonary valve syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2004;7:65–71.
4. Lakier JB, Stanger P, Heymann MA, Hoffman JI, Rudolph AM. Tetralogy of Fallot with absent pulmonary valve. Natural history and hemodynamic considerations. Circulation. 1974;50:167–175.
5. Prabhu MR. Trans-esophageal echocardiography for tricuspid and pulmonary valves. Ann Card Anaesth. 2009;12:167.
6. Jerath A, Roscoe A, Vegas A. Normal upper esophageal transesophageal echocardiography views. Anesth Analg. 2012;115:507–510.
7. Sivakumar K, Pavithran S, Coelho R. Unusual clinical presentation of rare adult survivors with a missing pulmonary valve associated with a missing left pulmonary artery. Pediatr Cardiol. 2013;34:1899–1902.

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