Melody® Transcatheter Pulmonary Valve (TPV) Therapy in a Patient with

Multiple Failed Right Ventricular Outflow Tract Repairs

Abstract

Background: A patient with a history of ventricular septal defect (VSD) and pulmonary atresia

presented for treatment of right ventricular outflow tract (RVOT) dysfunction. Previous attempts

at managing patient’s pulmonary atresia included Blalock-Taussig (BT) shunt placement for

palliation and early repair of the RVOT via Contegra graft (Medtronic, Inc.). Later, the Contegra

graft was replaced with a 19 mm homograft. This case report outlines the use of Melody®

Transcatheter Pulmonary Valve (TPV) Therapy (Medtronics, Inc.) to treat RVOT dysfunction.

Discussion: Melody TPV Therapy involves the placement of a heart valve made from a bovine

jugular vein attached to a stent. The construction integrates balloon expandable stent technology

with bovine valved conduit therapies (ie. Contegra). A catheter is used to guide the Melody

valve up to the heart and position it in the pulmonary conduit between the right ventricle and the

main pulmonary artery. The stent-valve apparatus is expanded with a balloon to its necessary

diameter, between 14 mm and 22 mm. The use of this therapy allows improved right ventricular

outflow in patients with failing conduits while avoiding open heart surgery.1

Conclusion: In the case of an 11-year-old patient with a history of pulmonary atresia and failure

or restenosis of previous conduit attempts, the Melody valve successfully provided a less

invasive alternative to open heart surgery with expectations for a high level of continued success.

The positive outcomes of this case support Bonhoeffer’s original assertion that transcatheter

valve replacement using a pre-stented valve would play an important role in the management of

conduit obstructions and pulmonary regurgitation.2 Extended follow-up of patient is needed to

further evaluate valve success.

Introduction

Pulmonary atresia is a congenital heart defect resulting from an improperly formed

pulmonary valve. As a result, the heart is unable to effectively pump deoxygenated blood from

the right ventricle into the pulmonary artery. Children born with pulmonary atresia typically

present with “blue baby syndrome,” or cyanosis, due to the lack of oxygen in the systemic

circulation.

Immediate palliative steps must be taken to enable circulation of blood through the lungs.

Due to its low risk and high effectiveness, the Blalock-Taussig (BT) shunt is most often selected

for palliation of pulmonary atresia.3 The BT shunt perfuses the lungs by providing a connection

between the carotid or subclavian artery and a side branch of the pulmonary artery; however, the

conduit is not large enough to sustain the heart forever. Generally, within a year of BT shunt

placement the malformed right ventricular outflow tract (RVOT) and pulmonary valve must be

repaired. The natural RVOT consists of a short tube-like extension of the right ventricle, thus a

conduit graft may be used to surgically replace the RVOT and pulmonary valve with a new and

functioning approximation of a RVOT and valve. Conduit grafts for the RVOT can be made

from a human RVOT and pulmonary valve (homografts) or from bovine or porcine vasculature

(xenografts), each with their own benefits and drawbacks.4 In cases of continued RVOT

dysfunction or stenosis, Melody Transcatheter Pulmonary Valve (TPV) therapy may be

performed to create a more structured conduit.1

Literature Review
 The first prototype for the Melody valve was introduced by Bonhoeffer in 2000.5 His

original 2002 study results concluded that this non-surgical method of pulmonary valve

replacement was not only possible, but resulted in no major complications in any of his eight

subjects.2 Since then, the technology has improved the lives of innumerable children with

congenital heart disease. A 2014 study evaluating the success rate of Melody valve placement at

follow-up found freedom from Melody valve dysfunction to be 96.9% at one year.6 A 2017

systematic review and meta-analysis reported a 96.2% procedural success rate for TPV

implantation, with a 4.1% rate of conduit rupture.7 Fraisse et al. report excellent follow-up of

RVOT function six months after Melody placement with a small concern for late-occurring

endocarditis which requires further research.8

Case Description

An 11-year-old female with history of congenital heart disease including ventricular

septal defect (VSD) and pulmonary atresia presented in June of 2017 with stenosis of her

pulmonary conduit. Patient was born with blue baby syndrome caused by pulmonary atresia and

underwent placement of a Blalock-Taussig shunt for palliation shortly after birth. Within the first

year of life the conduit was repaired with a Contegra graft. An echocardiogram in 2007 revealed

increased stenosis of the conduit and the Contegra graft was replaced by a homograft. During a

diagnostic cardiac catheterization in June of 2017 a 45 mmHg systolic pressure gradient was

measured between the right ventricle and pulmonary artery using a Swan-Ganz catheter

(Edwards). Angioplasty of the pulmonary conduit was performed using a 14 mm Atlas Gold

noncompliant balloon (Bard PV) inflated to 16 atm. The pressure gradient across the pulmonary

conduit was reduced to 20 mmHg before a slight tear in the conduit developed. Evaluation of the
remaining systolic gradient and the rigidity of the stenotic homograft resulted in a follow up

appointment for Melody TPV placement.

The Melody TPV therapy procedure was conducted in September of 2017. Initial

pressure measurements before valve placement were taken in the right side of the heart. The

existing homograft was evaluated as heavily calcified. A pressure gradient of 30 mmHg was

measured between the right ventricle and the pulmonary artery, roughly double the gradient

measured after balloon dilation in June. The homograft was dilated to widen the stenotic regions

using an 18 mm Atlas noncompliant balloon (Bard PV) at 10 atm (see Figure 1 & Figure 2).

Dilation was followed by pre-stenting of the conduit with a P3110 Palmaz stent (Cordis) (see

Figure 3). Post-dilation of the stent was performed with a 20 mm Atlas noncompliant balloon

(Bard PV). The Melody valve was crimped by hand onto the balloon from the Ensemble®

delivery system (Medtronic, Inc.) and positioned in the pre-stented conduit. The Ensemble

balloon-in-balloon system was used to fully expand the stent and Melody valve to fill the

existing homograft conduit. Pulmonary angiography revealed proper function of the Melody

valve (see Figure 4). A gradient of 10 mmHg was measured across the Melody valve post-

procedure. This gradient is acceptable and the Melody valve placement was considered

successful. No complications were reported during the procedure. The most recent

echocardiogram evaluations have shown minimal residual stenosis of the pulmonary conduit.
Figure 1. Pulmonary conduit stenosis prior to Figure 2. Pulmonary conduit stenosis reduced
dilation during Melody procedure. Image by balloon inflation during Melody procedure.
courtesy of the author. Image courtesy of the author.

Figure 3. Pre-stenting of pulmonary conduit Figure 4. Angiography of functioning

during Melody procedure. Image courtesy of Melody valve preventing backflow of blood
the author. into right ventricle. Image courtesy of the
author.
Discussion

Eight of every 5000 babies are born with congenital heart deformities involving the

RVOT and pulmonary valve. These deformities prevent the effective channeling of blood

through the right ventricle and into the pulmonary arteries, inhibiting the reoxygenation of blood

returning from the body.9

The Melody valve, constructed by Philipp Bonhoeffer in the late 90’s, consists of a

modified version of a balloon-expandable platinum-iridium stent with a segment of bovine

jugular vein sewn inside. This construction allows for TPV placement via a balloon expandable

valve. The Ensemble delivery system was developed to be used in conjunction with the Melody

TPV to ease introduction, delivery, and deployment of the valve.1 A balloon-in-balloon

angioplasty balloon, a 16 Fr long-sheath with a 22 Fr distal portion where the balloon is

mounted, and an introducer are included in the Ensemble. The Ensemble comes in three sizes

according to the outer balloon diameter - 18, 20, and 22 mm. Once the Melody valve is hand-

crimped to the desired balloon, the covering portion of the sheath is advanced over the valve to

provide protection and the delivery system is introduced into the body over a stiff guidewire.

Upon proper positioning in the right ventricular outflow tract, the inner and then outer balloon

are inflated and the valve is deployed. The Ensemble is removed after valve implantation and

deflation of the balloons. The functionality of the valve is assessed via pressure measurements

and contrast injection under fluoroscopy.1

The Melody valve must be placed inside an existing conduit. Bove et al. conducted a

retrospective study which found that 59% of Contegra patients had competent valve function

compared to 75% of patients receiving a homograft.4 Another retrospective study by Breymann

et al. revealed that Contegra conduit function over a 27-month follow-up was at least as good as
the function of homograft conduits.10 A 2011 assessment of the Contegra conduit as a risk factor

for graft replacement found that patient receiving Contegra were more than twice as likely to

undergo re-operation for graft placement as those receiving a homograft.11 As seen in this case

study and supported by previous literature, RVOT conduits can fail and do so more frequently

than desired. Regardless of homograft or xenograft conduit composition, the Melody TPV and

Ensemble deployment system provide a safe, effective, and less-invasive alternative to open

heart surgery for patients.

Conclusion

In the case of an 11-year-old patient with a history of pulmonary atresia and failure, or

restenosis, of previous conduit attempts, the Melody valve successfully provided a less invasive

alternative to open heart surgery. Analysis of previous literature leads to expectations of a high

level of continued RVOT patency. The positive outcomes of this case support Bonhoeffer’s

original assertion that transcatheter valve replacement using a pre-stented valve would play an

important role in the management of conduit obstructions and pulmonary regurgitation.2

Sustained positive results are expected based on previous research regarding Melody valve

success, however extended follow-up of patient is needed to further evaluate valve sustainability.
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