Case 42

Catheter Implantation of Stented

Pulmonary Valve
Louise Coats, Sachin Khambadkone, and Philipp Bonhoeffer

Age: 22 years
Gender: Female
Occupation: Student
Working diagnosis: Tetralogy of Fallot with pulmonary atresia

HISTORY TOF, has detrimental effects on RV function2 and exercise

capacity3 and can increase the risk of arrhythmia and sudden
This patient presented in early infancy when she was noticed death.4 Most often it is found in patients with prior trans-
to be cyanosed by a home health visitor. Following investiga- annular patch repair of classic TOF. The presence of branch
tion, the diagnosis of pulmonary atresia with VSD was made. pulmonary artery stenosis in this situation increases the regur-
Palliative surgery, namely bilateral placement of modified BT gitant load on the RV and is associated with accelerated clinical
shunts, was performed in infancy and early childhood to deterioration.5
enhance pulmonary blood flow.
This surgery permitted the patient to develop and grow nor- CURRENT MEDICATIONS
mally such that definitive surgical repair could then be per-
formed. At the age of 6, restoration of continuity between the None
RV and pulmonary arteries was established with a 17-mm
homograft conduit. A further 11-mm homograft conduit was PHYSICAL EXAMINATION
used to augment and attach the left pulmonary artery to the
reconstructed outflow tract. The VSD was closed. The operation BP 110/70 mm Hg, HR 74 bpm, oxygen saturation 97%
was uncomplicated, but recovery was slow with a long period
in intensive care. Following surgery, the patient had an unevent- Height 155 cm, weight 73 kg, BSA 1.77 m2
ful childhood. Surgical scars: Median sternotomy and bilateral thoracotomy
At age 18, she started her university education and became
aware of increasing fatigue and breathlessness with moderate Neck veins: Not distended
exertion. Over the ensuing years the problem became more Lungs/chest: Clear to auscultation
noticeable. She returned for follow-up.
Heart: Regular rate and rhythm were present, with the apex
C o m m e n t s : Pulmonary atresia with VSD is a variant of beat displaced to the left. There was an RV heave. A 3/6
TOF, and is sometimes called complex pulmonary atresia. The ejection systolic murmur was heard at the left sternal edge
pulmonary blood supply is dependent initially on patency of radiating to the back and to the left axilla. Also, a 2/4 early
the ductus arteriosus, and later on the presence of aortopulmo- diastolic murmur at the left sternal edge was present.
nary collateral arteries.
The BT shunt, first performed in 1944 by Alfred Blalock and Abdomen: No abnormalities detected
Vivian Thomas, marked the beginning of rapid development in Extremities: No peripheral edema or cyanosis was seen.
surgery for ACHD. This progress has resulted in a marked
improvement in prognosis for patients with congenital heart
defects with more than 85% of patients now surviving into adult LABORATORY DATA
life.1
Hemoglobin 13.3 g/dL (11.5–15.0)
Hematocrit 40% (36–46)
CURRENT SYMPTOMS White cell count 10.2 × 109/L (4.5–13.5)
The patient has noticed increasing tiredness and shortness of Platelet count 257 × 109/L (150–400)
breath on moderate exertion. She did not complain of palpita- Sodium 140 mmol/L (134–145)
tions, light-headedness, or chest pains. Potassium 3.7 mmol/L (3.5–5.2)
Creatinine 0.63 mg/dL (0.6–1.2)
NYHA class: II Urea 3.5 mmol/L (2.5–6.5)
PT 31 sec (26–38)
C o m m e n t s : Here the symptoms most likely represent APTT 10.7 sec (9.9–12.5)
conduit failure—either conduit stenosis or regurgitation. Pul-
monary valve regurgitation, present in many patients with C o m m e n t s : Normal blood results.

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ELECTROCARDIOGRAM ter intervention is considered, this finding is encouraging as it
indicates the potential for device stability.

Catheter Implantation of Stented Pulmonary Valve

I aVR V1 V4

II aVL V2 V5

III aVF V3 V6

Figure 42-1  Electrocardiogram.

Findings
Heart rate: 77 bpm
Rhythm: Sinus rhythm
QRS axis: Right-axis deviation (+110°)
QRS duration: 150 msec
RBBB pattern

C o m m e n t s : QRS duration correlates with RV volume in

patients with repaired tetralogy. A QRS duration on the resting Figure 42-3  Lateral view.
ECG of at least 180 msec is a predictor of life-threatening ven-
tricular arrhythmia.6
Findings
Cardiomegaly with retrosternal filling consistent with RV
CHEST X-RAY hypertrophy and/or a retrosternal conduit. Surgical clip relat-
ing to previous shunt ligation. The lung fields are clear.

EXERCISE TESTING
Exercise protocol:
Peak workload:
Reason for stopping:
ECG changes:
Ramp protocol
90 Watts
Dyspnea and leg pain
RBBB, frequent ectopic beats

Rest Peak
Heart rate (bpm): 74 153
Percent of age-predicted max HR: 77
O2 saturation (%): 97 98
Blood pressure (mm Hg): 110/70 155/80
Figure 42-2  Posteroanterior projection. Peak Vo2 (mL/kg/min): 19
Percent predicted (%): 62
Findings Ve/Vco2: 32
Cardiothoracic ratio: 62% Metabolic equivalents: 6.9

Moderate cardiomegaly, cardiac shape consistent with RV
hypertrophy. The inapparent pulmonary trunk fits with the
diagnosis of tetralogy. Calcification of the homograft and left
pulmonary artery can be seen. There is a surgical clip relating
to previous shunt ligation. There is a right aortic arch (boot-
shaped heart). The lung fields are clear.
C o m m e n t s : Poor cardiopulmonary exercise capacity
identifies ACHD patients at risk of hospitalization or death.7
Cardiopulmonary exercise capacity can improve following res-
toration of pulmonary valvar competency.8,9

C o m m e n t s : CXR is useful for identifying the presence of

circumferential homograft/conduit calcification. If transcathe-

Cases in Adult Congenital Heart Disease  235

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 ECHOCARDIOGRAM echocardiography correlated with severe pulmonary regurgi­
tation as quantified by MRI. However, these measure-
Right-Sided Lesions / Tetralogy of Fallot

ments should be interpreted with caution, as they can be

influenced by RV end-diastolic dysfunction (restriction).

MAGNETIC RESONANCE IMAGING

Figure 42-4  Two-dimensional apical four-chamber view.

Findings
A moderately dilated, hypertrophied RV was seen with mildly
impaired function. The LV was normal except for paradoxical
septal motion. There was trivial tricuspid regurgitation, with a
peak regurgitant velocity of 3.8 m/sec. There was mild dilation
of the RA. The IVC collapsed normally with respiration.
Figure 42-6  Two-dimensional single slice to b��–steady-state
��������������������������
free preces-
sion cine image, four-chamber view.
Findings
The main pulmonary artery measured 17 mm at its narrowest
point. The left pulmonary artery measured 3 mm proximally and
11 mm distally. The pulmonary regurgitant fraction was 20%.

C o m m e n t s : There was evidence of RV volume overload,

Ventricular Volume Quantification
although the central venous pressure was normal.
LV (Normal range) RV (Normal range)
EDV (mL) 131 (52–141) 171 (58–154)
ESV (mL) 51 (13–51) 70 (12–68)
SV (mL) 80 (33–97) 101 (35–98)
EF (%) 61 (56–75) 59 (49–73)
EDVi (mL/m2) 74 (65–99) 96 (65–102)
ESVi (mL/m2) 29 (19–37) 39 (20–45)
SVi (mL/m2) 45 (42–66) 57 (39–63)

C o m m e n t s : MR offers reliable quantitation of RV volume

and function. In this case, the degree of RV dilation is mild
despite the conduit dysfunction. RV function is normal.

Figure 42-5  Continuous-wave Doppler across the calcified homograft

demonstrating pulmonary regurgitant flow signal.

Findings
The calcified homograft was seen with degeneration of the
valve leaflets. The peak gradient was 45 mm Hg, with a mean
gradient of 21 mm Hg.
There was moderate pulmonary regurgitation lasting 71% of
the diastolic duration. The pressure half-time was 122 msec.
Diastolic flow reversal was present in the branch pulmo-
nary arteries, and there was a proximal left pulmonary artery
stenosis.

C o m m e n t s : The pressure half-time was less than

100 msec10 and duration of pulmonary regurgitation was less Figure 42-7  Three-dimensional contrast-enhanced angiogram of the right
than 77% diastolic duration11 on continuous-wave Doppler ventricular outflow tract and pulmonary arteries.

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Findings This showed a dimension of 18 mm × 21 mm, with a clear ste-
Mildly narrowed homograft conduit. Moderate proximal left notic segment in the center.

Catheter Implantation of Stented Pulmonary Valve

pulmonary artery stenosis. Mild kinking of the right pulmonary
artery origin.
C o m m e n t s : Care should be taken when measuring
dimensions from 3D MR angiography, as images are not ECG-
gated and are thus an average of the dimensions throughout
the cardiac cycle. In addition, signal dropout can occur in
regions of fast-moving blood, leading to possible overestima-
tion of stenoses. Nevertheless, 3D reconstruction provides
important additional information for planning of surgical or
interventional procedures.12
Significant pulmonary regurgitation was present with mod-
erate homograft obstruction and moderate stenosis of the
smaller homograft joining to the left pulmonary artery.
C o m m e n t s : This image is necessary for sizing the RVOT
in consideration for percutaneous PVR. Percutaneous pulmo-
nary valves can be implanted into outflow tracts up to 22 mm
in diameter. Balloon sizing is commonly performed in border-
line cases to document the distensibility of the potential site for
implantation.

CATHETERIZATION Focused Clinical Questions

and Discussion Points
Hemodynamics
Heart rate 75 bpm 1. Is there an indication for intervention in this patient?
Pulmonary regurgitation is the most important residual lesion in
Pressure Saturation (%) repaired TOF, correlating with RV size, exercise intolerance, and
RA mean 9 74 serious ventricular arrhythmias. Pulmonary valve replacement
RV 55/4 72 has beneficial effects on these parameters provided it is performed
PA 31/16 mean 21 66 before irreversible myocardial dysfunction occurs. Associated
LPA 16/6 mean 11 lesions, particularly branch pulmonary artery stenosis, aggravate
PCWP mean 10 pulmonary and tricuspid regurgitation, which further affect RV
Aorta 90/49 mean 63 98 size and function.
Cardiac output 4.27 L/min However, surgically implanted valves have limited longevity
Cardiac index 2.6 L/min/m2 and are likely to degenerate with time, committing patients to
further operations.13 The decision to operate should, therefore, be
Calculations
based on the balance between progressive ventricular dilatation,
Qp (L/min) 3.47
exercise intolerance, symptoms, arrhythmias, and that further
Qs (L/min) 4.63
reoperations will be needed.
Cardiac index (L/min/m2) 2.61 Prospective follow-up of this patient group with exercise testing
Qp/Qs 0.75 and assessment of RV size and function should better define the
PVR (Wood units) 3.17 natural history of the disease and provide firm guidelines for
SVR (Wood units) 11.66 timing especially in asymptomatic patients.14
The recent development of transcatheter alternatives may
Findings change the balance between medical and surgical intervention. It
Cardiac catheterization demonstrated severe homograft regur- may be that earlier, safer intervention will protect the RV from
gitation in addition to moderate left pulmonary artery stenosis. adverse remodeling. Thus the transcatheter approach may facil-
itate the growing inclination for earlier intervention without the
risks associated with repeat sternotomy and cardiopulmonary
bypass.
This patient has a combination of moderate conduit stenosis
and regurgitation and objective evidence of exercise intolerance
with gradual onset of symptoms of shortness of breath and
fatigue. These constitute criteria for elective intervention, albeit
RV (and LV) systolic function seem to be maintained at rest.
2. What are the options for intervention?
Surgical repair with a bifurcated homograft is one management
approach for this patient. She is symptomatic, with objective
evidence of exercise impairment and RV dilatation. However,
operative intervention must be undertaken with the understand-
ing that surgery may again be required in the future if homograft
degeneration occurs. As an alternative, a valved conduit using a
xenograft valve would be used in many centers in association
with a left pulmonary arterioplasty.
The conventional transcatheter management of this case
would involve placement of a left pulmonary artery stent and
a bare stent in the main pulmonary artery homograft to relieve
the pressure load on the RV. While this approach can improve
Figure 42-8  Lateral angiogram showing calcified homograft conduit with symptoms and reduce RV pressure,15 it is not optimal, as the
free pulmonary regurgitation. patient would continue to have free pulmonary regurgitation.
The third option is transcatheter PVR and stenting of the
Findings homograft left pulmonary artery stenosis. Though a newer
To assess the RVOT for potential percutaneous pulmonary technique, it has obvious advantages for the properly selected
valve implantation, a 25-mm PTS Sizing Balloon was inflated. patient.

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 3. What are the limitations of transcatheter valve replacement? INTERVENTION
Right-Sided Lesions / Tetralogy of Fallot

Percutaneous pulmonary valve replacement provides a novel The procedure was performed from a right femoral venous
transcatheter approach to this condition. The device is a bovine approach under general anesthesia. An ultra-stiff guide wire
jugular venous valve mounted inside a platinum iridium stent was stabilized in the distal right pulmonary artery to facilitate
that can be deployed in the same manner as a bare stent but with delivery of the device. The percutaneous pulmonary valve was
the additional benefit of restoring pulmonary valve competence. crimped onto a 22-mm Balloon in Balloon (BiB) delivery system.
The procedure has been performed in more than 700 patients The device was conveyed and deployed without complication
worldwide and has been associated with early clinical improve- in the narrowest portion of the main homograft. Subsequently,
ment, reduction in right ventricular volumes, and 98% freedom the wire position was changed to the left pulmonary artery and
from pulmonary regurgitation at 1 year.9 a 29/10 Genesis premounted stent on a 10-mm balloon was
Percutaneous pulmonary valve implantation is currently delivered to the smaller homograft conduit. There were no
limited to those patients whose RVOTs are not larger than 22 mm. immediate complications.
Furthermore, the presence of circumferential calcification or some
degree of stenosis is desirable, as it is likely to aid device stabil-
ity. At present, therefore, this procedure is rarely suitable for OUTCOME
patients who have pulmonary regurgitation in association with
patch augmentation of the RVOT, which was a common approach
to repair in the past. Other patients may be excluded if vascular
access is difficult, such as patients with azygos continuity of the
IVC, although the procedure can be performed via an internal
jugular approach. Documentation of coronary anatomy prior to
proceeding is essential as stent implantation can cause external
compression of a coronary artery if in close proximity (i.e., intra-
mural or intra-arterial course of the left anterior descending).
At present, although early results are promising, the long-term
fate of the valved stent remains unknown.9 Stent fracture can
occur and has been associated with clinical events such as in-
stent stenosis and stent migration in a minority.16 Nevertheless,
the procedure is less invasive and has fewer complications than
conventional surgery.17

Figure 42-10  Lateral angiogram post–percutaneous pulmonary valve

implantation showing relief of outflow tract obstruction and abolition of
regurgitation.

Findings
Hemodynamics postprocedure were:
Pressures
RV 46/10
PA 36/8 mean 20
LPA 23/8 mean 5
Aorta 116/63 mean 82

C o m m e n t s : In the postintervention pulmonary angio-

gram no regurgitation was seen through the valved stent. Per-
cutaneous pulmonary valve implantation has been successfully
combined with other transcatheter procedures such as ASD
closure and VSD closure.
Figure 42-9  Melody percutaneous pulmonary valve. (From Coats L, Tsang The patient experienced an early improvement in clinical
V, Khambadkone S, et al: The potential impact of percutaneous symptoms and was NYHA class I by 3 months. A progressive
pulmonary valve stent implantation on right ventricular outflow tract improvement in cardiopulmonary exercise capacity was noted.
re-intervention. Eur J Cardiothorac Surg 27:536–543, 2005.) 1 mo 1 yr
Pre post post
FINAL DIAGNOSIS Vo2 max (mL/kg/min) 19.0 24.3 27.1
AT (mL/kg/min) 10.4 11.8 14.1
Late homograft degeneration associated with pulmonary Workload (Watts) 90 130 125
regurgitation and RVOT obstruction Peak heart rate (bpm) 153 180 176
Proximal left pulmonary artery stenosis Peak blood pressure (mm Hg) 155/80 170/80 180/70
Respiratory exchange ratio 1.06 1.05 1.16
PLAN OF ACTION Echocardiography confirmed absence of pulmonary regurgi-
Percutaneous pulmonary valve implantation and left pulmo- tation with a peak gradient of 31 mm Hg (mean gradient
nary artery stent 16 mm Hg) across the percutaneous pulmonary valve and a

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peak velocity of 1.8 m/sec across the left pulmonary artery stent time analysis with a conductance catheter technique. Circulation
at 1 year. 95:643–649, 1997.

Catheter Implantation of Stented Pulmonary Valve

MRI at 1 year showed a substantial reduction in RV volume 6. Gatzoulis MA, Till JA, Somerville J, Redington AN: Mechanoelec-
(preprocedure values are shown in parentheses in the table trical interaction in tetralogy of Fallot. QRS prolongation relates
to right ventricular size and predicts malignant ventricular
below). arrhythmias and sudden death. Circulation 92:231–237, 1995.
7. Diller GP, Dimopoulos K, Okonko D, et al: Exercise intolerance in
Ventricular Volume Quantification adult congenital heart disease: Comparative severity, correlates,
LV (Prior data) RV (Prior data) and prognostic implication. Circulation 112:828–835, 2005.
8. Eyskens B, Reybrouck T, Bogaert J, et al: Homograft insertion for
EDV (mL) 131 (131) 137 (171) pulmonary regurgitation after repair of tetralogy of Fallot
ESV (mL) 49 (51) 55 (70) improves cardiorespiratory exercise performance. Am J Cardiol
85:221–225, 2000.
SV (mL) 82 (80) 82 (101)
9. Khambadkone S, Coats L, Taylor AM, et al: Transcatheter pulmo-
EF (%) 63 (61) 60 (59) nary valve implantation in humans—Initial results in 59 consecu-
tive patients. Circulation 112:1189–1197, 2005.
C o m m e n t s : Percutaneous pulmonary valve replacement 10. Silversides CK, Veldtman GR, Crossin J, et al: Pressure half-time
results in a reduction in RV volume and leads to an early predicts hemodynamically significant pulmonary regurgitation in
adult patients with repaired tetralogy of Fallot. J Am Soc Echocar-
improvement in symptoms and objective exercise capacity.9 diog 16:1057–1061, 2003.
11. Li W, Davlouros PA, Kilner PJ, et al: Doppler-echocardiographic
ACKNOWLEDGMENT assessment of pulmonary regurgitation in adults with repaired
tetralogy of Fallot: Comparison with cardiovascular magnetic
The MR images were contributed by Dr. Andrew Taylor, MRCP, resonance imaging. Am Heart J 147:165–172, 2004.
MD, FRCR. 12. Bogaert J, Dymarkowski S, Taylor AM: Clinical Cardiac MRI. Hei-
delberg, Germany, Springer-Verlag, 2005.
13. Stark J, Bull C, Stajevic M, et al: Fate of subpulmonary homograft
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insufficiency on ventricular function following repair of tetralogy 15. Fogelman R, Nykanen D, Smallhorn JF, et al: Endovascular stents
of Fallot. Evaluation using radionuclide ventriculography. J Thorac in the pulmonary circulation: Clinical impact on management and
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after complete repair of tetralogy of Fallot: Deleterious effects of systematic classification, and anticipatory management strategies
residual pulmonary regurgitation. Br Heart J 67:470–473, 1992. for stent fracture after percutaneous pulmonary valve implanta-
4. Gatzoulis MA, Balaji S, Webber SA, et al: Risk factors for arrhyth- tion. Circulation 115:392–397, 2007.
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Fallot: A multicentre study. Lancet 356:975–981, 2000. of percutaneous pulmonary valve stent implantation on right ven-
5. Chaturvedi RR, Kilner PJ, White PA, et al: Increased airway pres- tricular outflow tract re-intervention. Eur J Cardio-Thorac Surg
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pulmonary regurgitation after repair of tetralogy of Fallot. Real-

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