A Proposed Algorithm for Management of Patients With Congenital Cardiac Defects Associated With

8/2/2019 A Proposed Algorithm for Management of Patients With Congenital Cardiac Defects Associated With

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DAILY PRACTICE REVISED

A proposed algorithm for management of patients with congenital cardiac defects associated with

pulmonary hypertension

One of the objectives of the Pulmonary Hypertension Associated with Congenital Heart Disease

(PAH-CHD) Task Force is to bring up controversial issues to discussion. Two frequent problems in the

management of children with PAH-CHD are: 1) to define the need for cardiac catheterization; 2) to decide

which patients should undergo complete repair of the anomaly.

In developed countries, most children with congenital cardiac shunts are assigned to operation on

the basis of noninvasive evaluation only. This is based on the assumption that patients can be considered

as at low risk for immediate postoperative complications and late residual pulmonary hypertension ifsurgical treatment is offered early in life. Although this can be accepted as a general rule, early access to

treatment may not be the reality in many underserved areas. Furthermore, young babies with certain

anomalies (eg, truncus arteriosus, transposition of the great arteries and complete atrioventricular septal

defect) may develop severe pulmonary hypertension early during the first months of life. Some patients

with simple anomalies such as nonrestrictive ventricular septal defect or patent ductus arteriosus do not

have congestive heart failure or failure to thrive in their clinical history, thus indicating increased

pulmonary vascular resistance and absence of increased pulmonary blood flow.

Although there is general agreement that direct measurement of pulmonary vascular resistance is

necessary in some cases, there hasnt been evidence to support recommendations in terms ofcatheterization in children with congenital cardiac shunts and elevated pulmonary arterial pressure. There

hasnt been evidence to recommend closure of the septal defects with acceptable risk either.

In the Task Force, we attempted to develop a simple algorithm for patient management (see at the

end of the text). However, before using the algorithm in clinical practice, some considerations are

necessary:

1. There is no simple rule to assign children with PAH-CHD to catheterization or operation.

Decision must be individualized.

2. Decision should be based on multiple data obtained from the clinical history, physical

examination, oxymetry, qualitative and quantitative echocardiographic evaluation and cardiac

catheterization if necessary.

3. The criteria that appear in the algorithm are considerably strict. They were adopted as an

attempt to ensure a lower risk of postoperative (immediate and late) complications.

4. The algorithm is not applicable to complex situations as is the case of absent subpulmonary

ventricle. In this specific situation, even mild postoperative elevation of pulmonary vascular

resistance may result in failure of the circuit (cavopulmonary in this case).


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5. Criteria for patient assignment to temporary palliation (pulmonary arterial banding) are not

suggested in the algorithm.

An interesting point for discussion is the potential impact of the new drugs for pulmonary arterial

hypertension on the diagnosis-and- treatment algorithm of PAH-CHD. It is unlikely the availability of these

drugs will influence the decision to catheterize, in particular since noninvasive predictors of outcomes

have not been developed or validated for this patient population. However, the new therapies may

influence the decision to operate in two ways: 1) treatments may decrease the likelihood of residual

(persistent) pulmonary hypertension following repair of the defects in OPERABLE patients; 2) one could

THEORETICALLY make inoperable patients become operable. While these hypotheses remain to be

tested by means of randomized controlled studies, the possibility of combining medical and surgical

therapeutic strategies is suggested in the algorithm.

Antonio Augusto Lopes, MD, FPVRIDept. Pediatric Cardiology and Adult Congenital Heart Disease

The Heart Institute (InCor), University of So Paulo Medical School, So Paulo, Brazil

Vice-president for Education, Pulmonary Vascular Research Institute (PVRI)

Co-leader, Pulmonary Hypertension Associated with Congenital Heart Disease Taskforce, PVRI

Over the past decade, our ability to successfully perform corrective surgical repair in patients with

congenital heart defects and increased pulmonary vascular resistance has improved with advances in

anesthesiology, ICU support, development of therapeutic interventions and treatment modalities, (e.g.

inhaled nitric oxide, prostacyclin analogues and phosphodiesterase type 5 inhibitors), and utilization of

cardiac and pulmonary assist devices, ECMO, atrial septostomy and/or palliative Potts' shunt a(s needed

for acute pulmonary hypertension crises peri-operatively). However, just because we "can" carryout these

surgical repairs and discharge these patients from the hospital, it remains unclear if long term we are

doing what is in the best interests of the patients. We must remain cognizant of the natural history of

Eisenmenger Syndrome versus Idiopathic Pulmonary Arterial Hypertension. We must also consider the

adverse effects associated with the treatment options short and long term and their cost effects. Our

ability to carry patients through a "stormy" peri-operative period has significantly improved but long-termoutcomes remain unknown.

It is vital if we are to move forward to improve overall quality of life and long term outcomes for

children and adults with congenital heart defects who have increased pulmonary vascular resistance, we

need to evaluate the patients carefully and follow all the patients long term (i.e. at least 10-20 years and

preferably longer) whether they are repaired or not in order for us to determine if what "we are able to do"


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is "what we should be doing." "Benign Neglect", "Do no Harm" and "Better is the Enemy of Good" may be

preferable for some patients.

Factors other than resting pulmonary vascular resistance are important to consider when

assessing a patient for operability. Additional factors include:

1. Age of the patient

2. Pulmonary to systemic blood flow, i.e. Qp/Qs

3. Ratio of pulmonary to systemic vascular resistance, i.e. PVRI/SVRI

4 Associated anomalies, e.g. Down Syndrome, upper airway obstruction

5. Clinical characteristics, e.g. comorbid conditions, concomitant medications, altitude

6. Complexity of the congenital heart defect(s), i.e. simple versus complex congenital heart

defects

Robyn J. Barst, MD, FPVRIProfessor Emerita of Pediatrics Columbia University College of Physicians & Surgeons New York, New York, USA

Fellow, Pulmonary Hypertension Associated with Congenital Heart Disease Taskforce, Pulmonary Vascular Research

Institute (PVRI)

Co-Leader, Sponsored International Fellowships Taskforce, PVRI

LLR shuntingR shunting

CHF (pulmonary congestion)CHF (pulmonary congestion)

Failure to thriveFailure to thriveSAT OSAT O22 >95%>95% -- no gradient RUE vs. LEs (acyanotic defects)no gradient RUE vs. LEs (acyanotic defects)

PRESENTPRESENT ABSENTABSENT

OPERABLEOPERABLE

Postop. PH crises andPostop. PH crises and

residual PH may occurresidual PH may occur

in rare instancesin rare instances

Consider:Consider:

AgeAge

Type and size ofType and size ofthe defectthe defect

AssociatedAssociated syndromessyndromes

TGA (with or without VSD)TGA (with or without VSD)

Truncus arteriosusTruncus arteriosus

Complete AV septal defectComplete AV septal defect

NonNon--restrictive VSD, PDA,restrictive VSD, PDA,

aortopulmonary windowaortopulmonary window

CATHETERIZATIONCATHETERIZATION

Baseline PVRIBaseline PVRI


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A Proposed Algorithm for Management of Patients With Congenital Cardiac Defects Associated With

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