Paediatric Respiratory Reviews 13 (2012) 10–15

Contents lists available at ScienceDirect

Paediatric Respiratory Reviews

Mini-Symposium: Pulmonary Complications of Paediatric Systemic Disorders

Pulmonary Complications of Congenital Heart Disease

F. Healy 1, B.D. Hanna 2, R. Zinman 1,*
1
Division of Pulmonary Medicine, The Children’s Hospital of Philadelphia, USA
2
Division of Cardiology, The Children’s Hospital of Philadelphia, USA

EDUCATIONAL AIMS
 To discuss the variety of ways in which paediatric cardiac disease impacts the lung including anatomical and pathophysiological
mechanisms.
 To understand pulmonary oedema as it applies to the paediatric population, including the various pathophysiological mechanisms
and resultant effects on lung function.
 To emphasize the effects that cardiac surgery itself including cardiopulmonary bypass and direct trauma can have on pulmonary
structure and function.

A R T I C L E I N F O S U M M A R Y

Keywords: Cardiac and pulmonary pathophysiologies are closely interdependent, which makes the management of
Congenital heart disease patients with congenital heart disease (CHD) all the more complex. Pulmonary complications of CHD can
Lung function be structural due to compression causing airway malacia or atelectasis of the lung. Surgical repair of CHD
Pulmonary oedema
can also result in structural trauma to the respiratory system, e.g., chylothorax, subglottic stenosis, or
Fontan
diaphragmatic paralysis. Disruption of the Starling forces in the pulmonary vascular system in certain
Pulmonary hypertension
types of CHD lead to alveolar-capillary membrane damage and pulmonary oedema. This in turn results in
poorly compliant lungs with a restrictive lung function pattern that can deteriorate to cause hypoxemia.
The circulation post single ventricle palliative surgery (the so called ‘‘Fontan circulation’’) poses a unique
spectrum of pulmonary pathophysiology with restrictive lung function and a low pulmonary blood flow
state that predisposes to thromboembolic complications and plastic bronchitis. As the population of
patients surviving post CHD repair increases, the incidence of pulmonary complications has also
increased and presents a unique cohort in both the paediatric and adult clinics.
ß 2011 Elsevier Ltd. All rights reserved.

INTRODUCTION
In the healthy subject, there is a close relationship between the
functions of the cardiovascular and respiratory systems such that
changes in the metabolic requirements of the body are rapidly
followed by changes in both cardiac output and minute ventilation.
However, in the presence of congenital anomalies of the circulation
this linkage is almost always broken. Under such circumstances,
the ability of the heart to increase systemic and/or pulmonary
blood flow is often limited, arterial PO2 may be decreased by shunt
lesions, and O2 delivery cannot meet the needs of the tissues. Often
the circulatory derangement also places stress on the respiratory
system itself, causing signs and symptoms that mimic primary
respiratory disease. Pathologies in both systems frequently coexist
and impact each other, making patient diagnosis and management
more challenging.1
Because congenital defects of the circulation are so common
(approximately 7 infants per 1000 live births are affected) and the
alterations in respiratory function are often so profound, it is
essential that the physician interested in respiratory diseases be
well acquainted with the spectrum of CHD and the ways in which it
is manifested in infants and young children.
Direct pulmonary complications of CHD are either by structural
impact on the airways, abnormal pathophysiological mechanisms
leading to increased lung water and/or significant pulmonary
disease. Many children with CHD are at greater risk of infection
including respiratory tract infections, which can cause prolonged
hospitalization and delay of definitive cardiac repair.

ANATOMICAL COMPRESSION OF THE PAEDIATRIC AIRWAY

* Corresponding author. The Children’s Hospital of Philadelphia, Philadelphia, PA
19104. Tel.: +215 590 3749; Fax: +215 590 3500. This is an often unrecognized complication of CHD. A high index
E-mail address: zinman@email.chop.edu (R. Zinman). of suspicion must be maintained in infants with wheezing, stridor,

1526-0542/$ – see front matter ß 2011 Elsevier Ltd. All rights reserved.
doi:10.1016/j.prrv.2011.01.007
 F. Healy et al. / Paediatric Respiratory Reviews 13 (2012) 10–15
respiratory distress, apnoeic or cyanotic spells and atelectasis, which
can all be due to underlying tracheobronchomalacia. The aetiology
of airway obstruction can be divided into two groups:
Cardiac
This can occur in the setting of either cyanotic or acyanotic CHD.
Lesions that permit communication between the systemic and
pulmonary circulation and cause a large left-to-right shunt are the
most frequent congenital cardiac anomalies. These include ven-
tricular septal defect (VSD), patent ductus arteriosus (PDA), and
atrioseptal defect (ASD) as well as defects with similar pathophy-
siologic consequences but more complex anatomy, such as single
ventricle, aortopulmonary window, or truncus arteriosus. These
lesions are characterized by the recirculation of oxygenated blood
through the lungs and pulmonary vascular congestion. The shunt
produces excessive pulmonary blood flow and increased return of
pulmonary venous blood. Long-term complications then include left
ventricular diastolic dysfunction and dilated cardiomyopathy
causing airway compression. Enlargement of the left atrium, which
lies just below the carina can result in widening of the angle of
tracheal bifurcation with or without compression of the mainstem
bronchi. Large left-to- right shunts or mitral valve stenosis or
regurgitation may cause atrial enlargement. Combined enlargement
of the pulmonary arteries and left atrium is likely to result in left
bronchial compression.2 Massive cardiomegaly from underlying
cardiac disease can also compress the left main bronchus and in
some cases the left lower lobe of the lung itself can be compressed, a
common cause of left lower lobe bronchiectasis.
Vascular
These account for 1-1.6% of all CHD and include abnormalities
of the aortic arch, pulmonary artery slings, anomalous innominate
artery, and congenital absence of the pulmonary valve.3 These
lesions lead to compression of the airway by a pulsatile artery and
subsequent malacia of that airway. Although the clinical mani-
festations are usually the same, the possible combinations of
vascular abnormalities that can cause respiratory difficulty from
airway compression are virtually unlimited. Symptoms of airway
compression may be mild until a concurrent respiratory tract
infection exacerbates symptoms and prompts investigation. In
some instances, the diagnosis is not considered until there is
difficulty in extubating the child in the postoperative period.
A vascular ring is diagnosed when the trachea and oesophagus
are completely surrounded by an anomalous vascular structure
derived from the aortic arch. In pulmonary artery slings, the left
pulmonary artery arises from the right pulmonary artery and then
passes leftward between the trachea and esophagus. This can result
in compression of the right mainstem bronchus and distal trachea.
Complete tracheal rings can also be present in up to 50% of patients
with pulmonary artery slings. Other associated pulmonary mal-
formations include tracheomalacia, abnormal pulmonary lobulation
and bronchus suis.4 The clinical combination of pulmonary artery
sling, complete tracheal rings, and bridging bronchus is well
documented.5 In years past, the first approach to diagnosis was
the use of a barium oesophagogram. Although magnetic resonance
imaging is replacing that approach, the barium oesophagogram still
has an important role in the initial evaluation of infants and children
suspected of having airway compression by an anomalous vessel.
Pulmonary artery regurgitation during fetal life, owing to
congenital absence of the pulmonary valve, results in massive
enlargement of the pulmonary arteries and severe compression of
the trachea and main stem bronchi, causing lobar collapse or lobar
emphysema and severe respiratory distress. This lesion usually
occurs in association with tetralogy of Fallot.
11
Treatment of cardiovascular compression of the airways
Secondary malacia of the infant airway due to cartilage
destruction from extrinsic compression has been shown to occur
very early in infancy and resolve slowly.6 Early correction allows for
more normal tracheobronchial growth.2 In cases where compression
is due to associated CHD, simultaneous surgeries to repair the
cardiac anomaly and to relieve airway obstruction may be necessary.
Surgical repair is indicated in all patients with symptomatic vascular
rings and slings. Local stenosis of the trachea may be managed by
resection and reanastomosis or, in cases with longer segment
narrowing, a tracheoplasty may be required.
PULMONARY OEDEMA
Pathophysiology
Pulmonary oedema is caused by disturbance of the Starling
forces (hydrostatic and oncotic pressures) that dictate the flow of
water between capillaries and alveoli. Elevated hydrostatic forces
within the pulmonary capillaries increase the driving pressure of
fluid moving out of the capillary. This pressure disrupts the
integrity of the alveolar-capillary membrane causing capillary
stress fracture, which is visible on electron microscopy.7 As a
result, water starts to accumulate in the interstitium and alveoli,
causing pulmonary oedema.
Pulmonary oedema renders the alveoli unstable and it makes
the lungs stiff (decreases lung compliance), thereby increasing the
work of breathing to maintain adequate ventilation. There is
usually compression of small intraparenchymal airways by
engorged peribronchial vessels or by bronchial wall or peribron-
chial oedema (‘cuffing’) with wheezing (‘cardiac asthma’). Pul-
monary oedema, and the mechanical disturbances that it produces,
also impairs gas exchange resulting in hypoxemia.
Pulmonary oedema is a complication of many types of primary
cardiovascular disease that can be categorized as noted in Table 1.
Obstruction of pulmonary venous drainage increases capillary
pressure and results in transudation of fluid. Disease states that lead
to an increased right atrial pressure impair drainage of pulmonary
lymphatics resulting in fluid retention and often, pleural effusions
(right more than left). Left-to-right shunts increase pulmonary blood
flow leading to water retention in the lungs.
Table 1
Cardiovascular Diseases leading to Pulmonary Oedema
1. Pulmonary venous hypertension due to:
- Pulmonary veno-occlusive disease or
pulmonary vein stenosis
- Cor triatriatum
- Supra mitral ring
- Left ventricular dysfunction
- Transposition of the great arteries or
hypoplastic left heart with intact atrial septum
2. Decreased lymphatic flow:
- Lymphangiectasia
- Superior venacaval syndrome
- Single ventricle physiology
- Tricuspid valve stenosis
- Failing or stiff right ventricle
- Right ventricular outflow tract obstruction
3. Left-to-right heart shunts including:
- VSD
- ASD
- PDA
- Partial anomalous pulmonary venous
connection
- Systemic arteriovenous malformations
- Aorto-pulmonary connections including
surgical shunts
12 F. Healy et al. / Paediatric Respiratory Reviews 13 (2012) 10–15
Lung function in pulmonary oedema
Recent studies have demonstrated that even in the early phases
of pulmonary oedema perivascular cuffing negatively impacts the
lung parenchyma and causes decreased lung compliance.8 Spiro-
metry may reveal obstructive defects in cases of interstitial oedema
due to airway compression. As pulmonary oedema progresses and
the lungs stiffen, restrictive defects predominate. Total lung capacity
and diffusion capacity of carbon monoxide (DLCO) are abnormally
decreased in patients with acute or chronic pulmonary edema.9
Treatment of pulmonary oedema
Therapy should focus on treating the primary cardiovascular
problem with relief of pulmonary venous or lymphatic obstruction
and eliminating shunts. Diuretic therapy is effective in this setting
until definitive surgery is possible. The small airway disease of
‘cardiac asthma’ or single ventricle palliative physiology does
improve with inhaled bronchodilator treatments, as adjuncts to
diuresis.
Cardiopulmonary bypass and pulmonary oedema
In patients with CHD undergoing cardiopulmonary bypass
(CPB), systemic inflammatory responses contribute to pulmonary
oedema and resultant mechanical and gas exchange abnormal-
ities.10 Exposure of blood to artificial surfaces in the CPB circuit
activates host defense mechanisms, while splanchnic hypoperfu-
sion during CPB has been associated with intestine-derived
endotoxin passage into the circulation. Fluid priming of the CPB
circuit and the administration of cardioplegic solutions during
surgery also contribute to the systemic inflammatory response
during CPB.11 Treatment strategies employed to reduce this pro-
inflammatory reaction include the use of modified fluid gelatin to
maintain plasma oncotic pressures, heparin coated circuits to
inhibit complement activation, and modified ultrafiltration (MUF),
which removes plasma water and low molecular weight solutes.
PALLIATIVE SINGLE VENTRICLE PHYSIOLOGY
Palliative univentricular physiology (so-called ‘‘Fontan physiol-
ogy’’) is associated with passive, non-pulsatile drainage to the
pulmonary arteries. Patients typically have congested, small lungs
with a restrictive pattern of lung function (Section 9). The reason
why lungs are smaller is unclear but may be related to previous
pleurodesis in some cases. Alternatively, there may be an inherent
degree of pulmonary hypoplasia due to the lack of pulsatile flow to
potentially stimulate lung growth. There is also a high risk of
pulmonary embolic disease in this low flow state (Section 6).
Exercise capacity is significantly reduced in these patients due to
lower cardiac output, impaired increase of heart rate (chronotropy)
and ventilation-perfusion mismatch.12
This low flow state can also lead to the development of systemic
venous to pulmonary venous collateral vessels with progressive
cyanosis. In addition, in situations with underlying left pulmonary
artery stenosis, systemic arterial to pulmonary arterial collateral
vessels may also develop. The mechanism for the development of
collateral vessels in univentricular cases is unknown but has been
associated with increased levels of angiogenic growth factors.13
These collaterals can contribute to increasing cyanosis and an
element of right ventricular diastolic dysfunction with stiffening of
the ventricle walls. This may then in turn contribute to the
development of pulmonary oedema. Exclusion of hepatic blood
flow from the pulmonary blood flow also contributes to the
development of pulmonary arteriovenous malformations (AVM).
In cases of bilateral superior vena caval connections to the left and
right pulmonary arteries, the circulation on the opposite side to the
inferior vena cava has limited hepatic blood flow often resulting in
increased AVMs on the affected side.
Failing palliative single ventricle physiology
As this is a non-pulsatile system of passive flow there is a risk of
increasing pressures both in the arterial and venous pulmonary
circulation. This is a result of both systolic and diastolic ventricular
dysfunction. The use of a fenestration allows some relief of high
pressures by shunting blood flow from the high-pressure systemic
venous system into the right atrium. This in turn however, can
result in greater hypoxia. Overall, cardiac output will begin to
decline stimulating the release of hormones that act on the renal
tubules to produce fluid retention.
In addition, patients have a decreased oncotic pressure from the
hypoalbuminemia associated with protein losing enteropathy.
With end stage disease patients will also develop cardiac cirrhosis
further isolating hepatic factors from pulmonary flow and
contributing to AVM development.
Plastic Bronchitis
In this rare, potentially life-threatening condition patients
present with recurrent expectoration of branching, mucoid
bronchial casts, which vary in size from segmental casts of a
bronchus to casts filling the airways of an entire lung. Casts are
differentiated histologically as type 1, inflammatory casts com-
posed of fibrin and eosinophilic infiltrates and type 2, acellular
casts composed primarily of mucin and fibrin with no inflamma-
tory infiltrate.14 Type 2 casts are found in children with underlying
cardiac defects particularly post shunt procedures, Fontan being
the most common. In cases of failing palliative univentricular
physiology, the level of the dysfunction within the circuit should
be determined to guide therapeutic options, e.g., high pulmonary
vascular resistance/pulmonary artery stenosis, pulmonary vein
stenosis, atrioventricular valve dysfunction with secondary high
left atrial pressures and pulmonary venous congestion or
dysfunction of the systemic ventricle with secondary high left
atrial pressures and pulmonary venous congestion.
PULMONARY INFECTION
Respiratory tract infection in children with CHD is an important
cause of morbidity and mortality including respiratory failure,
prolonged mechanical ventilation, and hospitalization.15 These
patients often have many contributing factors that place them at
increased risk for respiratory tract infection (Table 2). Respiratory
viruses including respiratory syncytial virus (RSV), human
metapneumovirus, and influenza are commonly seen. In a study
of 2613 children under 24 months old with CHD, bronchiolitis was
the commonest cause of hospitalization (54.1%).16 RSV was the
most common agent identified and children receiving adequate
RSV prophylaxis had a 58.2% reduction in RSV related hospitaliza-
tion rate. The benefits of early corrective surgery after RSV
infection may not outweigh the risks of surgery. Cardiac surgery
Table 2
Risk factors for Respiratory Tract Infection in Congenital Heart Disease
 Severe underlying medical conditions
 Malnutrition
 Aspiration
 Prolonged duration of tracheal intubation and/or mechanical ventilation
 Use of antacids that influence gastric pH and allow oropharyngeal and
tracheal colonization
 Previous use of extended spectrum antibiotics that inhibit intestinal flora
 F. Healy et al. / Paediatric Respiratory Reviews 13 (2012) 10–15 13

performed during the symptomatic period of RSV infection has ary shunts (aortopulmonary and ventriculopulmonary) may
been associated with a high risk of postoperative complications produce septic pulmonary emboli.25
especially pulmonary hypertension (PH).17 For patients who
remain symptomatic and cannot be discharged, extending medical
PULMONARY HYPERTENSION
therapy may be more beneficial than early surgery, especially if
CPB is necessary for surgery.
A broad spectrum of cardiac and pulmonary diseases can lead to
the development of Pulmonary Hypertension (PH).1 Children with
Nosocomial Pneumonia
PH have pulmonary hypertensive crises precipitated by a rapid
increase in pulmonary vascular resistance (PVR) in response to
The incidence of nosocomial pneumonia (NP) in children after
stimuli including: hypercarbia, acidosis, and hypoxia. If PVR
cardiac surgery varies from 9.6% to 21.5%.18,19 Most studies
increases to the point where pulmonary artery pressure exceeds
demonstrate gram negative bacilli as the most common isolates in
systemic blood pressure, right ventricular diastolic and systolic
NP including Pseudomonas aeruginosa and Klebsiella pneumoniae
function decrease acutely and can rapidly progress to right
followed by fungi particularly Candida albicans.18 Candida is more
ventricular failure, arrhythmias, syncope, and death.
commonly seen after prolonged intensive care unit (ICU) stays and
nearly always after previous antibiotic treatment. Prior adminis-
Clinical presentation of pulmonary hypertension
tration of broad-spectrum antibiotics such as third-generation
cephalosporins, has become recognized as an important risk factor
Many of the clinical signs in PH are non-specific, e.g. dyspnoea,
for NP caused by antibiotic- resistant gram negative bacilli.20
weight loss, pallor or syncope. Association with cardiac symptoms
Rational use of antibiotics however, can shorten ICU stay and
such as chest pain or palpitations should raise the suspicion of PH.
reduce mortality and hospitalization expenses.
In children, careful physical examination may reveal a palpable
subxyphoid impulse indicating right ventricular hypertrophy. This
ATELECTASIS
clinical finding along with others such as hepatomegaly and
venous stasis should prompt the clinician to check a brain-type
Pathophysiology
natriuretic peptide level, a serum marker of ventricular dysfunc-
tion.26 In addition, cardiac consultation for further evaluation and
Atelectasis in patients with CHD can be attributed to extrinsic
management is appropriate at this stage of clinical suspicion.
compression from vascular malformation (section 1), restrictive
defects from pulmonary oedema (section 2) or from underlying
Management of pulmonary hypertension
respiratory tract infection (section 4). There are many post-
operative factors that place a patient at risk for the development of
Therapy of PH should be focused on increasing the capacity and
atelectasis.21 These include immobilization, splinting, cough
reducing the resistance of the pulmonary vascular bed thereby
suppression, mucus plugging and hypoventilation from pain and
decreasing right ventricular afterload. The presence of acidosis and
sedation. Neonates, who comprise a large population of patients
hypoxia in patients with underlying PH, if not treated, leads to
with CHD, are particularly sensitive to alveolar collapse because of
increased pulmonary vascular resistance and left ventricular
small airway size and reduced number of interalveolar pores of
compromise. Selective pulmonary vasodilator medications are
Kohn and bronchiole-alveolar channels of Lambert. Atelectasis
the current mainstay of PH therapy and include calcium channel
reduces lung compliance, increases work of breathing, and causes
blockers, prostanoids, endothelin antagonists, and phosphodies-
ventilation/perfusion mismatching that result in hypoxemia.
terase inhibitors.27 Caution is advised however, in cases where the
PH is due to downstream obstruction, such as pulmonary vein
COAGULATION DEFECTS
stenosis, pulmonary veno-occlusive disease, and left atrial
hypertension. Use of pulmonary vasodilators prior to the relief
Pulmonary Haemorrhage
of these obstructions can be associated with acute, life-threatening
pulmonary oedema.28 In addition, capillary obstruction that is not
CHD leading to Eisenmenger’s syndrome or PH is associated
reversible, e.g., alveolar-capillary dysplasia or capillary haeman-
with a paradoxical state of coexisting thrombotic and bleeding
giomatosis also result in acute pulmonary oedema. It is important
diathesis. In the presence of severe PH, pulmonary hemorrhage is
to recognize that in cases of PH, fluid boluses will not improve left
life-threatening. Haemoptysis has been reported as the cause of
ventricular performance or blood pressure and will in fact further
death in 11-30% of patients, though it can be self-limiting.22,23 It
impair right ventricular function. Ventilation strategies should aim
should be noted that pulmonary haemorrhage clears on chest
to recruit lung volume and increase capacitance of the pulmonary
radiograph usually within 72 hours and may therefore be
bed without overdistending the airspaces. Gastroesophageal reflux
differentiated from infectious consolidation.
and recurrent aspiration are often important contributing factors
that must be identified and managed effectively.
Pulmonary embolism

Thrombosis of the right-sided circulation and chronic pulmon-
ary embolic disease is a rare but often fatal complication of
congenital cardiac surgery particularly single ventricle palliative
procedures with an incidence of 5%-16%.24 The pulmonary blood
flow after these procedures is passive and dependent on the
transpulmonary gradient between the pulmonary artery and left
atrium. Elevated central venous pressure and slow right atrial
blood flow increase the risk of venous and right atrial thrombosis.
Hypoxia, implantation of foreign materials, and lesions that
involve high velocity blood flow all predispose to endocarditis
and thromboembolic complications. Infected systemic to pulmon-
SURGICAL TRAUMA IMPACTING THE RESPIRATORY SYSTEM
Despite advances in surgical interventions for CHD, complica-
tions from injury to surrounding structures remain a significant
risk (Table 3). Congenital cardiac surgery has become the most
common cause of chylous pleural effusion or chylothorax in
tertiary pediatric hospitals with an incidence of 3.8%.29 The
incidence of diaphragmatic paralysis due to phrenic nerve injury
during CHD surgery is up to 10% in certain studies.30–32 Close
monitoring with early detection and treatment of complications
often prevents prolonged ventilation and hospitalization, e.g.,
14 F. Healy et al. / Paediatric Respiratory Reviews 13 (2012) 10–15

Table 3
Surgical Trauma to the Respiratory System
Pulmonary Complication
Chylothorax
Recurrent laryngeal nerve injury
Diaphragmatic paralysis
Subglottic stenosis
Table 4
Lung Function Abnormalities in Congenital Heart Disease
Lung function abnormality
Obstructive lung disease
Restrictive lung disease
Diffusion impairment
Aetiology
Direct injury to the thoracic
duct or smaller vessels
High central venous pressures
Central vein thrombosis
Surgery involving the ductus
arteriosus, descending aorta
or left pulmonary artery
Manipulation of right common
carotid artery or internal jugular
vein for ECMO
Direct trauma (section of the
phrenic nerve)
Stretching of the phrenic nerve
Disruption of blood supply to
the phrenic nerve
Direct compression by the
endotracheal tube or cuff causing
oedema and ischaemia
Pathophysiology
Peribronchial cuffing
Airway compression
Vocal cord paralysis
Infection (bronchiolitis)
Chest wall deformity
Impaired lung growth
Reduced lung compliance
Diaphragmatic paralysis
Reduced pulmonary vascular bed
Atelectasis
Pulmonary oedema
suggests that earlier repair during the period of active postnatal
lung growth in the first few years of life may have less of an impact
on alveolar growth.
PATHOPHYSIOLOGY OF RESPIRATORY MANIFESTATIONS
From a clinician’s point of view, respiratory complications of
cardiovascular anomalies are characterized by the physical
findings. Frequently there is an increase in the amount of work
the respiratory system must do to maintain adequate ventilation.
The mechanisms responsible for the increase in the work of
breathing vary depending on the mechanical alterations caused by
each cardiovascular anomaly.
Most patients with a large-to-left right shunt or a left
ventricular obstruction will develop pulmonary oedema. As a
result, there is an increase in the amount of force that the
respiratory muscles have to generate to overcome the elastic recoil
of the lungs. Under these circumstances, intercostal and subcostal
retractions develop and the respiratory pattern tends to become
rapid and shallow. Moreover, the patient frequently attempts to
preserve lung volume by closing the glottis at the end of expiration.
In the face of severe pulmonary oedema, inflammation or infection
there can be the additional findings of nasal flaring and grunting.
When airway obstruction is the predominant respiratory
symptom, the obstruction is almost always intrathoracic and as
such is exacerbated during exhalation. Therefore, in children with
direct compression of the trachea and large bronchi by enlarged
vessels or heart chambers, or by narrowing of the small airways by
oedema the respiratory pattern tends to be slower and deeper, and
the physical examination reveals prolonged expiration, wheezing,
and pulmonary hyper-inflation. When the airway compression by
an anomalous vessel is severe, inspiratory stridor is also present,
indicating the relatively fixed nature of the obstruction.

dietary changes in chylothorax or plication of diaphragmatic
paralysis in univentricular cases.21
PULMONARY FUNCTION TESTING
Restrictive lung function appears to be a prominent finding in
many types of CHD both pre and post surgical repair.33–35 In
addition, both obstructive and diffusion defects can also be seen
depending on the underlying pathophysiology (Table 4). A study of
52 patients post Fontan procedure at a median follow up of 10
years demonstrated restrictive lung function with reduced forced
vital capacity (FVC), forced expiratory volume in one second (FEV1)
and FEV1/FVC in 58% of patients.34 These abnormalities may be
intrinsic to the underlying CHD but may also in part be sequelae of
therapies including surgery. Potential iatrogenic causes include
diaphragmatic paralysis, restrictive thoracic cage from sternot-
omy, CPB, and respiratory muscle weakness. In a study of 46
children undergoing ASD repair, FVC and expiratory reserve
volume were lower in those that had a sternotomy with CPB
compared to those that had percutaneous device closure.36 This
suggests that interventions themselves may have an unexpected
negative impact on lung function and all potential therapies should
be carefully considered. However, in cases of pulmonary over-
circulation, e.g., VSD and ASD, surgical correction of excessive flow
has been shown to improve the mechanical properties of the lungs
including resistance of the respiratory system.37–39 Earlier repair of
CHD has been associated with improved lung function and exercise
capacity.34,36,40 Gaultier et al demonstrated a significant decrease
in vital capacity (VC) and lung compliance in groups of children
with tetralogy of Fallot repaired at mean ages of 4 and 5 years
compared to those repaired at a mean age of one year.40 This
CONCLUSION
To completely assess the clinical impact of CHD one must
consider the effect on pulmonary physiology. Disruption of the
Starling forces within the vascular system will disturb pulmonary
vascular physiology with resultant abnormalities in lung function.
Earlier repair of CHD during the period of active postnatal lung
growth in the first few years of life may have less of an impact on
alveolar growth and function. As the number and age of patients
surviving post CHD repair increases, the incidence of pulmonary
complications is increasing and presents a unique cohort in both
the paediatric and adult clinics. Ongoing studies are needed to
define the approach to management of the complex pulmonary
complications in this population.
CONFLICT OF INTEREST STATEMENT
The authors of this manuscript have no relevant conflicts of
interest to declare.
RESEARCH DIRECTIONS
 Approach to the management of atelectasis in the patient
with congenital heart disease.
 Effect of different types of congenital cardiac lesions (hypo
versus hyperperfusion of the lungs) on lung function in
both the pre and postoperative phase.
 Guidelines on the management of postoperative pulmon-
ary complications in paediatric patients with CHD
including diaphragmatic paralysis and recurrent laryngeal
nerve injury.
 F. Healy et al. / Paediatric Respiratory Reviews 13 (2012) 10–15
PRACTICE POINTS
 Anatomical compression of the paediatric airway due to
cardiac disease or vascular malformation can be diagnosed
early and in most cases is treatable.
 Diuretics are important in the treatment of pulmonary
oedema but overall focus should be on treating the
primary cardiovascular problem with relief of pulmonary
venous or lymphatic obstruction and eliminating shunts.
 Patients with surgical single ventricle palliative physiol-
ogy present a unique spectrum of pulmonary complica-
tions including restrictive lung function, thromboembolic
disease, protein losing enteropathy, and plastic bronchitis.
 Families should be educated about the potential pulmon-
ary complications of congenital cardiac surgery and
physicians should be vigilant for signs of these including
infection, atelectasis, chylothorax, diaphragmatic paraly-
sis, and recurrent laryngeal nerve injury.
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