Flap valved closure of ventricular septal defects with increased pulmonary

vascular resistance
Prasanna Simha Mohan Rao, Varadaraju Raju and Madhusudana Narayana
Interact CardioVasc Thorac Surg 2010;11:577-580; originally published online Aug 19,
2010;
DOI: 10.1510/icvts.2010.243865

The online version of this article, along with updated information and services, is
located on the World Wide Web at:
http://icvts.ctsnetjournals.org/cgi/content/full/11/5/577

Interactive Cardiovascular and Thoracic Surgery is the official journal of the European Association
for Cardio-thoracic Surgery (EACTS) and the European Society for Cardiovascular Surgery
(ESCVS). Copyright © 2010 by European Association for Cardio-thoracic Surgery. Print ISSN:
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 ARTICLE IN PRESS

doi:10.1510/icvts.2010.243865

Editorial
New Ideas
Interactive CardioVascular and Thoracic Surgery 11 (2010) 577–580
www.icvts.org

Institutional report - Congenital

Progress Report
Flap valved closure of ventricular septal defects with increased

Work in
pulmonary vascular resistance
Prasanna Simha Mohan Rao*, Varadaraju Raju, Madhusudana Narayana

Protocol
Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bannerghatta Road, Bangalore, India

Received 21 May 2010; received in revised form 27 July 2010; accepted 29 July 2010

Institutional
Report
Abstract

Closure of ventricular septal defect (VSD) in children with elevated pulmonary vascular resistance (PVR) is associated with significant
morbidity and mortality with pulmonary hypertensive (PH) episodes being a major postoperative problem. Flap valved closure of VSD is
reported to decrease morbidity and mortality. We report our experience of closure of a VSDs in patients with severe PH, using a valved

Article
ESCVS
patch in an effort to reduce the risk of operation. Eighteen consecutive patients with a large VSD with severe PH (mean PVR )8 Wood
units) underwent flap valved closure (as described by Novick et al.) of VSD during a one-year study period. The mean age at surgery was
8.3"3.9 years (range: 3–13 years). Mean PVRI was 13.02"4.05 Wood units. In-hospital 30-day mortality was 5.6% (1y18). Mechanical

Proposal for Bail-

out Procedure
ventilation time averaged 11.6"8.1 hours. Postoperative pulmonary artery pressures were significantly reduced. Four patients had PH crisis
postoperatively. Obvious opening and closing of the flap valve was detected by echocardiography in eight patients. There were no late
deaths due to cardiac causes. Closure of a large VSD in patients with severe pulmonary hypertension could be performed with low morbidity
and mortality when a flap valve patch was used.
䊚 2010 Published by European Association for Cardio-Thoracic Surgery. All rights reserved.

Negative
Results
Keywords: Ventricular septal defect; Pulmonary vascular resistance; Flap valve patch; Cardiopulmonary bypass; Low cardiac output

1. Introduction tively is especially difficult with a high mortality and

Follow-up
morbidity. We report our experience of closure of a VSDs

Paper
Congenital heart disease with septal defects and a large in patients with severe PH, using valved patch in an effort
left to right shunt often causes pulmonary artery (PA) to reduce risk of operation.
hypertension. The course of the disease is variable and

State-of-the-art
depends on the size of the defect, magnitude of the left
to right shunt, and the pulmonary vascular response to 2. Patients and methods
increased pulmonary blood flow and pressure. PA pressure
is hyperkinetic in the early phase but may eventually
become a fixed elevation due to a fixed increase of 2.1. Patients

Best Evidence
pulmonary vascular resistance (PVR) w1x. They clinically
manifest with decreased exercise tolerance, cyanosis, con- Between August 2008 and July 2009, 18 patients present-

gestive cardiac failure, often hemoptysis, and finally death
(Eisenmenger’s syndrome).
Closure of a ventricular septal defect (VSD) with increased
PVR is associated with significant morbidity and mortality
w2, 3x. Acute congestive heart failure, pulmonary hyperten-
sive (PH) crisis, and acute respiratory failure are the
principal causes of postoperative death. In some patients
the postoperative increase of PVR may be more marked,
Topic
ing to our unit with a VSD and increased PVRI (mean PVRI
)8 Wood units) were included in the study. The patients
were evaluated preoperatively by history and physical
Nomenclature
examination, chest radiography, electrocardiography,
transthoracic echocardiography (TTE), and cardiac
catheterization.
Complete hemodynamic data were determined by cardiac
catheterization. Pulmonary and systemic flow and resis-
Historical

may regress slowly, may show intermittent spontaneous or tance were measured at rest and 100% oxygen. Color
Pages

precipitated crisis-like exacerbation, and may require pro-
longed ventilation and specific strategies to maintain a
lower PVR w4x.
Many patients in developing nations come at a stage of
M-mode echocardiography was done to assess the direction
of shunting across VSD. The decision for surgery was based
on the presence of any augmentation of left to right shunt
or increase in pulmonary vascular blood flow following
Communication

near eisenmengerization and their management periopera- sublingual nitrate administration on preoperative echocar-
Brief

diography even if the patients showed an unfavorable PVR

*Corresponding author. Shri, No 33, 2ndy3rd Cross, Sampige Road, Malles-
waram, Bangalore 560003, Karnataka, India. Tel.: q91 93 41216294. on formal hemodynamic testing in the cardiac catheteri-
E-mail address: prasannasimha@gmail.com (P.S. Mohan Rao). zation laboratory. The study was approved by the Hospital
Case Report

䊚 2010 Published by European Association for Cardio-Thoracic Surgery

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 ARTICLE IN PRESS
578 P.S. Mohan Rao et al. / Interactive CardioVascular and Thoracic Surgery 11 (2010) 577–580
Research Ethics Committee, and written informed consent
was obtained from the parents of each child.
2.2. Surgical procedure and cardiopulmonary bypass
Surgery was performed with standard cardiopulmonary
bypass (CPB) with moderate hypothermia and cold blood
cardioplegia was used in all patients. Inj phenoxybenzamine
(0.5 mgykg i.v.) was given prior to CPB. Zero balance ultra
filtration was done throughout CPB. The operative approach
was transatrial.
A patch was constructed using the technique described by
Novick et al., using a Gore-Tex patch approximately as
large as the defect to be closed. A fenestration that was
half of the expected aortic annulus diameter was made in
the patch. Following this, a separate flap patch at least
2 mm larger than the fenestration was constructed and was
sewn onto the superior margin of the fenestration along
one-third of the circumference. A separate tethering stitch
was placed at the inferior apex of the flap valve and tied
loosely over a Hegar dilator that was the same size as the
fenestration. This tethering stitch length approximated the
diameter of the fenestration. The VSD patch was sewn into
place orienting the patch in such a way that the flap valve
was placed on the left ventricular (LV) side with the flap
opening towards the LV apex to avoid subaortic obstruction.
Patent foramen ovale (PFO) was left open in all patients.
Tricuspid valve competence was checked and repaired if
necessary.
2.3. Weaning from CPB
Prior to discontinuation of CPB, dobutamine 3 mgykgymin,
nitroprusside 0.5–2 mgykgymin, and the phosphodiesterase
inhibitor milrinone 0.35 mgykgymin were started. Inj phen-
oxybenzamine 0.5 mgykgyday were given as infusion for
48 h followed by oral formulation. Left atrial (LA) line was
placed if required and systemic vasoconstrictors were start-
ed through it. After weaning from CPB, modified ultrafil-
tration was used in all the patients. Epicardial echo-
cardiography was used to assess the degree of shunting
across the fenestration.
2.4. Postoperative management
Pulmonary and systemic artery pressures were monitored
continuously. Mild to moderate hypocarbia was maintained
during mechanical ventilation. Serial postoperative TTE was
used to assess the degree of shunting across the fenestra-
tion. Once the patients are fully awake and hemodynami-
cally stable, they were extubated. Inhaled nitroprusside
and milrinone nebulisation were given intermittently (every
2 hours) or when PA pressure surges occur. Inotropes were
weaned-off once patients were hemodynamically stable.
Milrinone infusion was stopped within 36–48 hour provided
PA pressure surges stopped or no right to left shunting
across PFO. Oral phenoxybenzamine was administered at
48 hours and sildenafil (1 mgykg 4 hourly) was adminis-
tered on the first postoperative day (POD). Patients were
monitored for seven days in ICUyintermediate care for PH
crisis.
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2.5. Follow-up
All patients were followed-up at regular intervals (every
three months) with clinical examination, and TTE to esti-
mate the PA pressure, assess the right ventricular function
and to demonstrate any right-to-left shunting.
2.6. Statistical analysis
All data are expressed as mean"standard deviation. Con-
tinuous variables at baseline and following surgery were
compared with unpaired t-test. A P-value of 0.05 or lower
on any test was considered to indicate statistical signifi-
cance. Data were analyzed using SPSS software (SPSS Inc,
Chicago, IL, USA).
3. Results
3.1. Patient characteristics
The mean age at surgery was 8.3"3.9 years (range: 3–
13 years) and female to male ratio was 1.25:1. One patient
with PVR 13.3 Wood units had Down’s syndrome.
3.2. Preoperative data
A perimembranous VSD was diagnosed in 80% of patients.
Muscular VSD was seen in 20% of patients. Cardiac cathe-
terization showed the ratio of systolic pulmonary artery
pressure to systolic systemic arterial blood pressure ranged
from 0.8 to 0.9 (mean: 0.85). Mean PVR was 13.02"4.05
Wood units (range: 8.5–23 Wood units) and after oxygen
administration was 8.34"2.5 Wood units. PVR was more
than 10 Wood units in 11 patients and was )15 Wood units
in four patients. The ratio of pulmonary artery flow to
systemic arterial blood flow (QpyQs) ranged from 0.5 to
2.0 (mean: 1.44"0.36) and increased to a mean of
1.91"0.34 after oxygen administration, and was -1.5 in
eight patients. Mean pulmonary artery pressure (MPAP)
ranged from 57 to 80 mmHg (mean: 68.8"9.7 mmHg) and
was )70 mmHg in nine patients. Arterial oxygen saturation
ranged from 88 to 99% (mean: 95.2"3.04%). Preoperative
catheterization data are summarized in Table 1.
3.3. Operative data
Eighteen patients underwent flap valved closure of VSD
out of which four patients could not be weaned-off CPB
initially and required placement of LA line, through which
systemic vasoconstrictors (noradrenaline and vasopressin)
were started and then patients could be weaned from CPB.
Table 1. Preoperative catheterization data
Variables Mean value
PVRI 13.02"4.05
QpyQs 1.44"0.37
SpO2 (%) 95.2"3.04
PAsyAos 0.88"0.5
PAsyAos, ratio of pulmonary artery systolic pressure to aortic systolic pres-
sure; PVRI, pulmonary vascular resistance index; QpyQs, ratio of pulmonary
blood flow to systemic blood flow; SpO2 , arterial hemoglobin oxygen
saturation.
 ARTICLE IN PRESS
P.S. Mohan Rao et al. / Interactive CardioVascular and Thoracic Surgery 11 (2010) 577–580
One patient with PVR of 9.3 Wood units had a persistent
rise in PA pressure )70% of systemic pressure, inspite of
maximizing pulmonary vasodilators and had significant LV
dysfunction. She had her chest kept open and was shifted
to the ICU.
3.4. Postoperative period
Mechanical ventilation time ranged from 8 to 36 hours
(mean: 11.6"8.1 hours). Three patients with a PVR of
18.43, 8.8 and 15 Wood units had an episode of PH crisis
on the day of surgery and were managed with administra-
tion of inhaled pulmonary vasodilators, oxygen administra-
tion, and support with inotropic and vasodilator drugs.
One patient with PVR of 9.3 Wood units required splinting
open of the sternum. She had persistent elevation of the
PA pressure in the postoperative period and developed
repeated episodes of PH crisis on first POD. She developed
intractable supraventricular tachycardia followed by low
cardiac output (LCO) and expired on the second POD.
Three patients with a PVR of 18.43, 9.3 and 18.6 Wood
units, respectively went into supraventricular tachycardia
on the day of surgery and were managed with administra-
tion of amiodarone as a bolus followed by infusion. Six
patients developed right lower lobe collapse of which three
required pleural drain insertion for concomitant effusion
and two required bronchoscopy. One patient with PVR of
18.43 Wood units had an episode of PH crisis on the third
POD, which was triggered by right lower lobe collapse due
to bronchomalacia requiring reintubation. The patient
underwent tracheostomy on the eighth POD and was in the
ICU for one month and was later discharged at their
request. She expired after 48 days due to bronchomalacia
which required stenting but her parents refused treatment
for it. The patient with Down’s syndrome could not be
weaned-off the ventilator due to upper airway obstruction
caused by a large tongue. He required a tracheo-
stomy on the second POD. Take down of tracheostomy was
done on 10th POD and he was discharged on the 14th POD.
The other patients had an uneventful postoperative course.
3.5. Echocardiographic assessment
Eight patients had a right-to-left shunt through the valved
patch as determined by TTE (eight patients up to the
second POD, one patient on the third POD and no patients
up to the seventh POD). Three patients had right to left
shunting across the PFO. Postoperative TTE results are
summarized in Table 2. All patients had preserved right
ventricular function (by visual estimation on echocardio-
graphy) and none of the patients had significant tricuspid
regurgitation.
Table 2. Postoperative echocardiographic assessment
Time No right to left shunt Right to left shunt
(cases) (cases)
Day 2 10 8 PAsyAos
Day 7 17 0 SpO2
Three months 16 0
Six months 16 0 MPAP, mean pulmonary artery pressure; PAsyAos, ratio of pulmonary artery
Nine months 14 0 systolic pressure to aortic systolic pressure; SpO2 , arterial hemoglobin oxygen
Downloaded from icvts.ctsnetjournals.org by on October 20, 2010
579
Editorial
3.6. Clinical outcome
Thirty-day mortality was 5.6%. One patient with PVR of
New Ideas
18.43 Wood units expired after 48 days due to bronchom-
alacia. There have been no subsequent deaths on follow-
up. At a mean follow-up of nine months (range: five to
12 months), one patient with PVR of 13.56 Wood units had
Progress Report
TTE evidence of moderate PH at three months, postopera-
Work in
tively. Sildenafil and phenoxybenzamine were continued for
the next two months and were stopped when follow-up PA
pressure was persistently low. The others were asympto-
matic with normal or near normal PA pressure. Postopera-
Protocol
tive hemodynamics are given in Table 3.
We compared our data to a historical control of 18
patients with PVRI )8 Wood units, where the mortality
was 12.6% and the mean ventilation time was 86"12 hours.
Institutional
Report
4. Discussion and conclusions
Historically, surgical closure of a large VSD with severe
PH was associated with a high mortality rate w4, 5x. Even
Article
ESCVS
in the present era, postoperative PH remains a significant
risk factor for morbidity and mortality.
Acute congestive heart failure, PH crisis, and acute res-
Proposal for Bail-
out Procedure
piratory failure are the principal causes of postoperative
death. CPB, infusion of protamine, and other factors that
could cause the release of vasoactive substances, such as
thromboxane A2 and catecholamines could result in pul-
monary vasoconstriction and acute PH crisis w6, 7x.
Negative
Creation of an intracardiac shunt to prevent right ventri-
Results
cular failure has been used with success. Novick and col-
leagues used a flap valved double-patch for closure of VSDs
in children with increased PVR w3, 8x. The double patch
Follow-up
technique provides a simple physiological mechanism for
Paper
unloading the right ventricle during periods of severe PH,
whether acute and transient or sustained. The modification
allowed more patients to be extubated within 48 hours and
State-of-the-art
with lower operative mortality. The flap valve allows early
extubation and prevents supra-systemic pulmonary pres-
sures that can be rapidly fatal in the postoperative period
and allows patients to ride through PH crisis without the
need for prolonged intubation and multiple maneuvers
Best Evidence
albeit at the cost of transient desaturation, while main-
Topic
taining adequate systemic cardiac output which is tolerated
well until the PH crisis is resolved.
In our study, all patients were children with high PVR.
Thirty-day mortality was 5.6%. One patient with a PVR 9.3 Nomenclature
Wood unit died on the second POD with frequent episodes
of PH crisis and intractable supra ventricular tachycardia
followed by LCO. Early extubation was possible in most of
our patients thereby diminishing the need for prolonged
mechanical ventilation. In addition, prolonged stay in the
Historical
Pages
Table 3. Preoperative and postoperative hemodynamic status
Characteristics Before repair After repair P-value
Communication
MPAP (mmHg) 68.83"9.67 34.4"11.42 0.0001
0.88"0.5 0.52"0.2 0.013
Brief
95.2"3.04 97.9"2.4 0.005
saturation.
Case Report
 ARTICLE IN PRESS
580 P.S. Mohan Rao et al. / Interactive CardioVascular and Thoracic Surgery 11 (2010) 577–580
ICU is associated with an increase in septic complications
w9x and other adverse events. Early extubation reduces the
use of resources that are already limited in a number of
developing countries.
Severe reactive postoperative PH may occur despite a
technically successful operation and active conventional
management, including the administration of high inspired
oxygen, hyperventilation, sedation, neuromuscular block-
ade, and support with inotropic and vasodilator drugs w6x.
When these therapies fail, patients can be considered for
inhaled nitric oxide and extracorporeal life support (ECLS)
which ensures adequate oxygenation and systemic perfusion
in the face of a markedly elevated PVR and may allow time
for the PH crisis to resolve w10x.
PH after closure of a large VSD continues to cause signif-
icant morbidity and mortality even in industrialized coun-
tries. The use of nitric oxide and extra corporeal membrane
oxygenation rescue has reduced the mortality, but with
significant cost and patient morbidity. These expensive and
sophisticated modalities are not available in many countries
throughout the world w10x.
Patients with VSD with severe PH who would otherwise be
considered for lung transplantation can undergo conven-
tional cardiac surgery using flap valve method of VSD
closure in a significant subset. Reports show that patients
with PH improved their hemodynamics and exercise capa-
bility after closure of the septal defect. It implies that
pulmonary vascular remodeling might take place but
requires long-term follow-up w11x.
We conclude that a valved patch in cases of severe PH
can undergo operation with reasonable morbidity and mor-
tality in the absence of sophisticated pharmacological or
mechanical intervention.
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 Flap valved closure of ventricular septal defects with increased pulmonary
vascular resistance
Prasanna Simha Mohan Rao, Varadaraju Raju and Madhusudana Narayana
Interact CardioVasc Thorac Surg 2010;11:577-580; originally published online Aug 19,
2010;
DOI: 10.1510/icvts.2010.243865
This information is current as of October 20, 2010

Updated Information including high-resolution figures, can be found at:

& Services http://icvts.ctsnetjournals.org/cgi/content/full/11/5/577
References This article cites 10 articles, 5 of which you can access for free at:
http://icvts.ctsnetjournals.org/cgi/content/full/11/5/577#BIBL
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