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Tiêu đề Critical Care After Surgery for Congenital Cardiac Disease
Trường học Vietnam National University Hospital
Chuyên ngành Medical Sciences / Cardiology / Critical Care
Thể loại Chương
Thành phố Hà Nội
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383CHAPTER 36 Critical Care After Surgery for Congenital Cardiac Disease (Box 36 2) The details of the specific considerations for selected lesions are presented in their respective sections The initi[.]

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(Box 36.2) The details of the specific considerations for selected

lesions are presented in their respective sections

The initial assessment following cardiac surgery begins with a

review of the operative findings This includes details of the

op-erative repair and CPB, particularly total CPB time, myocardial

ischemia (aortic cross-clamp), and circulatory arrest or antegrade

perfusion times; concerns about myocardial protection; recovery

of myocardial contractility; typical postoperative systemic arterial

and central venous pressures; findings from intraoperative

trans-esophageal or epicardial echocardiography, if performed;

vasoac-tive medication requirements; and hemostatic management This

information guides subsequent examination, which should focus

on the quality of the repair or palliation plus a clinical assessment

of cardiac output (Box 38.3) In addition to a complete

cardio-vascular examination immediately upon arrival to the PICU, a

routine set of laboratory tests should be obtained, including a

chest radiograph, 12- or 15-lead electrocardiography (ECG),

blood gas analysis, serum electrolytes and glucose, ionized

calcium and lactate measurements, complete blood count, and

coagulation profile

This information, together with an understanding of the

preoperative and postoperative loading conditions of the heart, are

essential for clinical management in the immediate postoperative period Optimizing preload involves more than just giving volume

to a hypotensive patient There are numerous considerations to fluid balance involving types of isotonic fluid, ultrafiltration in the operating room, optimal hematocrit, and the use of diuretics or vasopressors Fluid itself can be detrimental if excess extravascular water results in interstitial edema and end-organ dysfunction of vital organs such as the heart, lungs, and brain Occasionally, per-mitting a right-to-left shunt at the atrial level can optimize preload

to the left ventricle in some conditions (discussed later) Maintain-ing aortic perfusion after CPB and improvMaintain-ing the contractile state

of the heart with higher doses of catecholamines are reasonable goals, but they may have particularly deleterious consequences in the newborn myocardium after hypothermic CPB The benefits of afterload reduction are well known but, if excessive, may result in hypotension, coronary insufficiency, and cardiovascular collapse Pacing the heart can stabilize the rhythm and hemodynamics, but

it also may contribute to dyssynchronous, inefficient cardiac con-traction or may induce other arrhythmias Although lifesaving in many instances, mechanical support of the failing myocardium in the form of extracorporeal life support (ECLS) or ventricular assist devices has its own set of limitations and morbidities Almost every treatment approach has its own set of adverse effects Supporting cardiac output in the postoperative patient is a balance between the promise and poison of therapy

Monitoring

The goal of postoperative monitoring following cardiac surgery primarily focuses on assessing the adequacy of circulatory status and oxygen delivery The level of vigilance in the immediate post-operative period can be optimized by the combination of labora-tory values and physical examination findings with data obtained via noninvasive devices and invasive monitoring to assess intracar-diac pressures and oxygen saturations

Near-infrared spectroscopy (NIRS) provides a noninvasive, continuous estimate of regional oxygen supply and demand that serves as a surrogate for hemodynamics and cerebral and somatic oxygenation It is based on the differential absorption of varying wavelengths of light by hemoglobin as it associates with oxygen to measure oxygen content in a localized tissue bed.22 While data demonstrating a definitive impact on overall outcome are lacking, there are reports that correlate decreased cerebral and/or somatic NIRS with increased mortality, prolonged length of stay (LOS), and worsening neurologic outcomes.23 , 24

Invasive monitoring of central venous pressure is routine for most patients following cardiac surgery Intracardiac or transtho-racic left atrial (LA) catheters are often used to monitor patients after complex reparative procedures Pulmonary arterial (PA) catheters now are seldom used but may be particularly useful if one anticipates postoperative pulmonary hypertension, allowing rapid detection of pressure changes and assessment of the response to interventions

LA catheters are especially helpful in the management of pa-tients with ventricular dysfunction, coronary artery perfusion abnormalities, or mitral valve disease The mean LA pressure typically is 1 to 2 mm Hg greater than mean right atrial (RA) pressure, which generally varies between 1- and 6-mm Hg in nonpostoperative pediatric patients undergoing cardiac catheter-ization In postoperative patients, mean LA and RA pressures are often greater than 6 to 8 mm Hg However, they generally should

be less than 15 mm Hg The compliance of the right atrium is

•  BOX 36.2 Ten Intensive Care Strategies to Diagnose

and Support Low–Cardiac Output States

1 Know in detail the cardiac anatomy and its physiologic consequences.

2 Understand the specialized considerations of the newborn and

implica-tions of reparative rather than palliative surgery.

3 Diversify personnel to include experts in neonatal and adult congenital

heart disease.

4 Monitor, measure, and image the heart to rule out residual disease as a

cause of postoperative hemodynamic instability or low cardiac output.

5 Maintain aortic perfusion and improve the contractile state.

6 Optimize preload (including atrial shunting).

7 Reduce afterload.

8 Control heart rate, rhythm, and synchrony.

9 Optimize heart-lung interactions.

10 Provide mechanical support when needed.

•  BOX 36.3 Signs of Heart Failure or Low–Cardiac

Output States

Signs

Cool extremities/poor perfusion

Oliguria and other end-organ failure

Tachycardia

Hypotension

Acidosis

Cardiomegaly

Pleural effusions

Monitor and Assess

Heart rate, blood pressure, intracardiac pressure

Extremity temperature, central temperature

Urine output

Mixed venous oxygen saturation

Arterial blood gas pH and lactate

Laboratory measures of end-organ function

Echocardiography

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greater than that of the left atrium except in the newborn; thus,

pressure elevations in the right atrium of older patients with two

ventricles typically are less pronounced Possible causes of

abnor-mally elevated LA pressure are listed in Box 38.4

In addition to pressure data, intracardiac catheters in the right

atrium (or a percutaneously placed central venous catheter), left

atrium, and pulmonary artery can be used to monitor the oxygen

saturation of systemic or pulmonary venous blood and indicate

the presence or absence of atrioventricular synchrony Following

reparative surgery, patients with no intracardiac shunts and

ade-quate cardiac output may have a mild reduction in RA oxygen

saturation to approximately 60% Lower RA oxygen saturation

does not necessarily indicate low cardiac output If a patient has

arterial desaturation (complete mixing lessons, lung diseases, and

so on), the arteriovenous oxygen saturation difference is normally

,30% Hence, even a low RA saturation may be in keeping with

appropriate oxygen delivery and extraction Elevated RA oxygen

saturation often is the result of left-to-right shunting at the atrial

level (e.g., from the left atrium, anomalous pulmonary vein, or

left ventricular [LV]-to-RA shunt) Blood in the LA normally is

fully saturated with oxygen (i.e., approximately 100%) The two

chief causes of reduced LA oxygen saturation are an atrial-level

right-to-left shunt and pulmonary venous desaturation from

ab-normal gas exchange

In the absence of left-to-right shunts, PA oxygen saturation is

the best representation of the “true” mixed venous oxygen

satura-tion because all sources of systemic venous blood should be

thor-oughly mixed as they are ejected from the RV When elevated, this

saturation is useful in identifying residual significant left-to-right

shunts following repair of a VSD The absolute value of the PA

oxygen saturation is a predictor of significant postoperative

re-sidual shunt In patients following TOF or VSD repair, PA

oxy-gen saturation greater than 80% within 48 hours of surgery with

a fractional inspired oxygen concentration (Fio2) less than 0.5 is a

sensitive indicator of significant left-to-right shunt (Qp/Qs 1.5)

1 year after surgery.25

Low Cardiac Output Syndrome

Although low cardiac output after CPB is often attributable to

residual or undiagnosed structural lesions, progressive low–cardiac

output states do occur A number of factors have been implicated

in the development of myocardial dysfunction following CPB,

including (1) the inflammatory response associated with CPB,

(2) myocardial ischemia from aortic cross-clamping, (3)

hypother-mia, (4) reperfusion injury, (5) inadequate myocardial protection,

and (6) ventriculotomy (when performed) The typical decrease in

cardiac index has been well characterized in newborns following an

arterial switch operation (ASO).26 In a group of 122 newborns, the

median maximal decrease in cardiac index that typically occurred

6 to 12 hours after separation from CPB was 32%, and 1 in 4 of these newborns reached a nadir of the cardiac index lower than

2 L/min per square meter.26 Anticipation of this low cardiac out-put syndrome (LCOS) and appropriate intervention can do much

to avert morbidity or the need for mechanical support Mixed ve-nous oxygen saturation, whole-blood pH, and lactate are labora-tory measures commonly used to evaluate the adequacy of tissue perfusion and, hence, cardiac output

Volume Adjustments

After CPB, the factors that influence cardiac output—such as preload, afterload, myocardial contractility, heart rate, and rhythm—must be continuously assessed and manipulated as needed Volume expansion (increased preload) is commonly nec-essary, followed by appropriate use of vasoactive agents Atrial pressure and the ventricular response to changes in atrial pressure must be evaluated Ventricular response is judged by observing systemic arterial pressure and waveform, heart rate, skin color, peripheral extremity temperature, peripheral pulse magnitude, urine output, core body temperature, and acid-base balance

Preserving and Creating Right-to-Left Shunts

Selected children with low cardiac output may benefit from strat-egies that allow right-to-left shunting at the atrial level in the face

of expected postoperative RV dysfunction A typical example is early repair of TOF, when the hypertrophied and poorly compli-ant right ventricle may be further compromised by increased volume load from pulmonary regurgitation secondary to a trans-annular patch on the RV outflow tract These children will benefit from leaving the foramen ovale patent to permit right-to-left shunting of blood, thus preserving cardiac output and oxygen delivery despite the attendant transient cyanosis When the fora-men ovale is not patent or is surgically closed, RV dysfunction can lead to reduced LV filling, low cardiac output, and, ultimately, LV dysfunction In infants and neonates with repaired truncus arte-riosus, the same concerns apply and may even be exaggerated if

RV afterload is elevated because of pulmonary hypertension

Other Strategies

Additional strategies to support low cardiac output associated with cardiac surgery in children include the use of atrioventricular pacing for patients with complete heart block or prolonged inter-ventricular conduction delays and asynchronous contraction.27

Appreciation of the hemodynamic effects of positive and negative pressure ventilation may be used to assist cardiac output Avoid-ance of elevated body temperatures and even inducing hypother-mia along with appropriate sedation and even paralysis may provide end-organ protection during periods of low cardiac out-put and aid in the management of postoperative arrhythmias, such as junctional ectopic tachycardia

Mechanical Cardiac Support

Perioperative mechanical circulatory support (MCS) can be life-saving for critically ill children and young adults with CHDs.28 , 29

The most common form of pediatric MCS is extracorporeal membrane oxygenation (ECMO) ECMO can be used as rescue during extracorporeal cardiopulmonary resuscitation (ECPR), in failure to wean from CPB, or in patients who develop low–cardiac output states postoperatively despite high levels of pharmacologic support.30 , 31 it is presently less commonly used as a bridge to heart

•  BOX 36.4 Common Causes of Elevated Left Atrial

Pressure After Cardiopulmonary Bypass

Decreased ventricular systolic or diastolic function

Left atrioventricular valve disease

Large left-to-right intracardiac shunt

Chamber hypoplasia

Intravascular or ventricular volume overload

Cardiac tamponade

Arrhythmia

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transplantation—it is more often used as a prelude to long-term

mechanical assist devices or simply to allow time for

decision-making.29 , 32 An analysis of 96,596 operations from 80 centers

reporting to the Society of Thoracic Surgeons Congenital Heart

Surgery Database showed that MCS was used in 2.4% of cases.33

Children who underwent Norwood procedures (17%) or

com-plex biventricular repairs (14%) were more likely to receive MCS

Substantial variation exists in MCS rates across both high- and

low-volume centers Overall, 53% of those children who received

MCS did not survive to hospital discharge, with mortality greater

than 70% for certain operative lesions (truncus arteriosus repair,

Ross-Konno operation).33 Despite this high mortality, it is

impor-tant to recognize that survival would have been virtually zero for

those children without MCS

In a recent report from the Pediatric Cardiac Critical Care

Consortium Registry,34 of the 14,526 eligible medical as well as

surgical cardiac ICU hospitalizations, 449 (3.1%) had at least one

ECMO run Of these, 329 (3.5%) were surgical and 120 (2.4%)

were medical hospitalizations Of the surgical group, 33 (10%)

included preoperative ECMO only and 296 (90%) included

post-operative ECMO Overall, in-hospital mortality was 48.9% in

the surgical group and 63.3% in the medical group; mortality

rates for hospitalizations including ECPR were 82.7% (surgical)

and 50% (medical).34

Due to improved technology, reliability, and mechanical

dura-bility of devices, higher rates of patient survival, reduced adverse

events, and limited availability of organs for transplantation,

intra-corporeal MCS has become an accepted long-term inpatient and

outpatient therapy for those with advanced heart failure awaiting

heart transplantation.35 Ventricular assist devices (VADs) can

sup-port function of the left ventricle (left ventricular assist device

[LVAD]), right ventricle (right ventricular assist device [RVAD]),

or both (biventricular assist device [BiVAD]) A total artificial

heart (TAH) replaces the heart itself VADs have two different

mechanisms of blood flow: pulsatile or continuous Continuous

flow devices contain an impeller that rotates at high speed to

pro-pel blood These include axial flow impro-pellers (e.g., HeartMate II

[Thoratec Corp.]), or centrifugal pumps (e.g., HeartWare HVAD

[HeartWare Corp.]) Paracorporeal pulsatile devices (e.g., Thoratec

PVAD-BiVAD [Thoratec Corp.], Berlin Heart EXCOR BiVAD

[Berlin Heart Corp.]) are also used in selected cases Guidelines

exist with regard to CPR in children with MCS.35 A more detailed

discussion of MCS can be found in Chapter 28

Right Ventriculotomy and Restrictive Physiology

Right ventricular restrictive physiology has been demonstrated by

echocardiography as persistent anterograde diastolic blood flow

into the pulmonary circulation following reconstruction of the

RV outflow in infants and children This occurs in the setting of

elevated RV end-diastolic pressure and RV hypertrophy when the

right ventricle demonstrates diastolic dysfunction with an

inabil-ity to properly relax and fill during diastole The poorly compliant

RV usually is not dilated in this circumstance, and pulmonary

valve regurgitation is limited because of the elevated RV diastolic

pressure.36 , 37

The term restrictive RV physiology is also commonly used in the

immediate postoperative period in patients who have a stiff,

poorly compliant, and sometimes hypertrophied RV The elevated

ventricular end-diastolic pressure restricts filling during diastole,

causing an increase in RA filling pressure and, ultimately, systemic

venous hypertension Because of the phenomenon of ventricular

interdependence, changes in RV diastolic function and septal position in turn affect LV compliance and function Factors con-tributing to diastolic dysfunction include lung and myocardial edema following CPB, inadequate myocardial protection of the hypertrophied ventricle during aortic cross-clamp, coronary ar-tery injury, residual outflow tract obstruction, volume load on the ventricle from a residual VSD or pulmonary regurgitation, and dysrhythmias In many centers, a residual atrial communication is left to mitigate the perioperative sequelae associated with restric-tive RV physiology, namely, a low–cardiac output state In such a scenario, patients may be desaturated following surgery (typically

in the 75% to 85% range) because of this right-to-left shunting, but they maintain systemic cardiac output while avoiding significant systemic venous hypertension As RV compliance and function improve (usually within 2 to 3 postoperative days), the amount of shunt decreases and both anterograde pulmonary blood flow and Sao2 increase

If significant restrictive RV physiology develops in the absence

of an unrestrictive atrial communication, a low–cardiac output state with increased right-sided filling pressure (usually 12 mm Hg) ensues Such patients often have cool extremities, oliguria, and metabolic acidosis As a result of the elevated RA pressure, hepatic congestion, ascites, increased chest tube output, and pleu-ral effusions may be evident These patients may be tachycardic and hypotensive, with a narrow pulse pressure Preload must be maintained despite an already elevated RA pressure Significant inotropic support often is required (typically, epinephrine 0.05–0.1 µg/kg per minute) A phosphodiesterase inhibitor, such

as milrinone, can be beneficial because of its lusitropic properties; however, one must be cautious in the use of these agents with renal impairment.38 Sedation and paralysis often are necessary for the first 24 to 48 hours to minimize energy expenditure and as-sociated myocardial work Factors that further impair ventricular diastolic filling—such as loss of AV synchrony, accumulation of pleural fluid or ascites, and high tidal volume ventilation with air trapping—should be mitigated early in the postoperative course Mechanical ventilation, either hypoinflation or hyperinflation

of the lung, hypothermia, and acidosis can contribute to increased afterload on the right ventricle and pulmonary regurgitation Synchronized intermittent positive-pressure ventilation with the lowest possible mean airway pressure should be the aim, as dis-cussed previously

Diastolic Dysfunction

Occasionally, there is an alteration of ventricular relaxation, an active energy-dependent process, which reduces ventricular com-pliance This is particularly problematic in patients with a hyper-trophied ventricle undergoing surgical repair, such as TOF or Fontan surgery, and following CPB in some neonates when myo-cardial edema may significantly restrict diastolic function.37 , 38 The poorly compliant ventricle with impaired diastolic relaxation has

a reduced end-diastolic volume and stroke volume b-Adrenergic antagonists and calcium channel blockers add little to the treat-ment of this condition In fact, hypotension or myocardial de-pression produced by these agents often outweighs any gain from slowing the heart rate Calcium channel blockers are relatively contraindicated in neonates and small infants because of their dependence on transsarcolemmal flux of calcium to both initiate and sustain contraction

A gradual increase in intravascular volume to augment ven-tricular capacity, in addition to the use of low doses of inotropic

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agents, is of modest benefit in patients with diastolic dysfunction

Tachycardia must be avoided and AV synchrony maintained to

optimize diastolic filling time and decrease myocardial oxygen

demands If low cardiac output persists despite treatment,

vasodi-lators can be carefully attempted to alter systolic wall tension

(afterload) and thus decrease the impediment to ventricular

ejec-tion Because the capacity of the vascular bed increases after

vaso-dilation, simultaneous volume replacement is often necessary A

noncatecholamine inodilator with vasodilating and lusitropic

(improved diastolic state) properties, such as milrinone, is useful

under these circumstances in contrast with other inotropic

agents.39

Pharmacologic Support

General principles of pharmacologic support in the neonatal and

pediatric patient center on the recognition of the developmental

limitations of the neonatal myocardium and the well-described

reductions in cardiac output 6 to 12 hours after separation from

CPB.26 Despite ongoing development and maturity of adrenergic

receptors and L-type calcium channels, catecholamine-based

ino-tropic agents and vasodilators are efficacious in this population

Other nonvasoactive agents serve as adjuncts to optimizing

post-operative hemodynamics and fluid balance The combination of a

low-dose inotrope and an afterload reducing agent—or, more

commonly, a phosphodiesterase inhibitor—has been shown to

decrease the occurrence of postoperative LCOS following CPB.39

It should be understood that the need for vasoactive and

ino-tropic support varies greatly among patients recovering from

car-diac surgery and even over time for an individual patient

progress-ing through the postoperative care continuum The intensity of

pharmacologic support employed must be constantly evaluated

One must not embrace a false sense of security when caring for a

patient with adequate cardiac output when this requires

dispro-portionate pharmacologic support The current approach is to

employ the lowest level of inotropic and vasoactive support

neces-sary for the achievement of hemodynamic goals Due to advances

in preoperative stabilization, anesthetic strategies, surgical

tech-nique, myocardial protection, and CPB, it is not uncommon for

a patient to return to the PICU requiring only low doses of

milrinone or epinephrine or no pharmacologic support at all A

detailed discussion of cardiovascular pharmacology is beyond the

scope of this chapter but can be found in Chapter 31

Managing Acute Pulmonary Hypertension

in the Intensive Care Unit

Children with many forms of CHD are prone to perioperative

elevations in pulmonary vascular resistance (PVR).40 This

situa-tion complicates the postoperative course when transient

myocar-dial dysfunction is further challenged by increased RV afterload.41

Although postoperative patients with pulmonary hypertension

often are presumed to have active and reversible pulmonary

vaso-constriction as the source of their pathophysiology, the intensivist

is obligated to explore anatomic causes of mechanical obstruction

that impose a barrier to pulmonary blood flow Elevated LA

pres-sure, pulmonary venous obstruction, branch pulmonary artery

stenosis, or surgically induced loss of the vascular tree all raise RV

pressure and impose an unnecessary burden on the right heart

Similarly, a residual or undiagnosed left-to-right shunt raises

pul-monary artery pressure postoperatively and must be addressed

surgically In these cases, the use of pulmonary vasodilator strate-gies augments only residual or undiagnosed shunts and increases the volume load on the heart

Several factors peculiar to CPB may raise PVR: pulmonary vascular endothelial dysfunction, microemboli, pulmonary leu-kostasis, excess thromboxane production, atelectasis, hypoxic pulmonary vasoconstriction, and adrenergic events all have been suggested to play a role in postoperative pulmonary hypertension Postoperative pulmonary vascular reactivity has been related not only to the presence of preoperative pulmonary hypertension and left-to-right shunts but also to the duration of total CPB The threat of postoperative pulmonary hypertensive crises can be partially addressed by providing surgery at earlier ages, pharmaco-logic interventions, and other postoperative management strate-gies (Table 36.1)

Pulmonary Vasodilators

Nitric oxide (NO) is the mainstay of therapy in patients with pulmonary hypertension requiring critical care NO is a vasodila-tor formed by the endothelium from l-arginine and molecular oxygen in a reaction catalyzed by NO synthase NO then diffuses

to the adjacent vascular smooth muscle cells, where it induces vasodilation through a cyclic guanosine monophosphate-depen-dent pathway.42 , 43 Because NO exists as a gas, it can be delivered

by inhalation to the alveoli and, hence, to the adjacent blood sels Once it diffuses across the wall of the pulmonary blood ves-sels, NO enters the vascular lumen There, it is rapidly inactivated

by hemoglobin, resulting in selective pulmonary vasodilation Inhaled NO (iNO) has advantages over intravenously adminis-tered vasodilators that may cause systemic hypotension and in-crease intrapulmonary shunting Inhaled NO lowers pulmonary artery pressure in a number of diseases without the unwanted ef-fect of systemic hypotension This efef-fect is especially dramatic in children with cardiovascular disorders and postoperative patients with pulmonary hypertensive crises.41 , 44 , 45

Therapeutic uses of iNO in children with CHD abound in the ICU Newborns with total anomalous pulmonary venous connec-tion (TAPVC) frequently have obstrucconnec-tion of the pulmonary venous pathway where it connects anomalously to the systemic

TABLE 36.1 Critical Care Strategies for Postoperative Treatment of Pulmonary Hypertension

Anatomic investigation Residual anatomic disease Opportunities for right-to-left

shunt as pop-off Intact atrial septum in right heart failure Sedation/anesthesia Agitation/pain

Moderate hyperventilation Respiratory acidosis Moderate alkalosis Metabolic acidosis Adequate inspired oxygen Alveolar hypoxia Normal lung volumes Atelectasis or overdistension Optimal hematocrit Excessive hematocrit Inotropic support Low output and coronary perfusion Vasodilators Vasoconstrictors/increased afterload

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venous circulation When pulmonary venous return is obstructed

preoperatively, pulmonary hypertension is severe and requires

urgent surgical relief Use of iNO in this or other settings of

sus-pected pulmonary venous obstruction is contraindicated On the

other hand, increased neonatal pulmonary vasoreactivity,

endo-thelial injury induced by CPB, and remodeling of the pulmonary

vascular bed in this disease contribute to postoperative pulmonary

hypertension Postoperatively in the patient with TAPVC after

adequate surgical relief of obstruction, iNO dramatically reduces

pulmonary hypertension without adverse changes in heart rate,

systemic blood pressure, or vascular resistance

Postoperative patients with TAPVC, congenital mitral stenosis,

and other pulmonary venous hypertensive disorders associated

with low cardiac output are among the most responsive to iNO

These infants are born with significantly increased amounts

of smooth muscle in their pulmonary arterioles and venules

Histologic evidence of muscularized pulmonary veins and

pulmo-nary arteries suggests the presence of vascular tone and capacity

for change in resistance at both the arterial and venous sites The

increased responsiveness to iNO seen in younger patients with

pulmonary venous hypertension may result from pulmonary

va-sorelaxation at a combination of precapillary and postcapillary

vessels Resolving the primary venous obstruction is of utmost

importance before using iNO in these lesions

Several groups have reported successful use of iNO in a variety

of other congenital heart defects following cardiac surgery

Inhaled NO is especially helpful when administered during a

pulmonary hypertensive crisis.46 Successful iNO use has been

described after the Fontan procedure, following late VSD repair,

and with a variety of other anatomic lesions for which patients are

at risk of developing postoperative pulmonary hypertensive

cri-ses.45–47 Oxygen saturation in response to iNO generally does not

improve in very young infants who are excessively cyanotic after a

bidirectional Glenn anastomosis.48 In these cases, increasing

car-diac output and cerebral blood flow will have a much greater

impact on arterial oxygenation because elevated pulmonary

vascu-lar tone is seldom the limiting factor in the hypoxemic patient

after the bidirectional Glenn operation.49

Inhaled NO can be used diagnostically in neonates with RV

hypertension after cardiac surgery to discern those with

revers-ible vasoconstriction In patients with Ebstein anomaly, a

clini-cal response to iNO can accurately differentiate between

func-tional and anatomic pulmonary atresia.50 In addition, the use of

iNO in such patients can facilitate anterograde pulmonary

blood flow and hemodynamic stabilization Failure of the

post-operative newborn to respond to iNO should be regarded as

strong evidence of anatomic and possibly surgically remediable

obstruction.50

If iNO must be discontinued before the pathologic process has

been resolved, hemodynamic instability can be expected The

withdrawal response to iNO can be attenuated by pretreatment

with the type V phosphodiesterase inhibitor sildenafil.51 Sildenafil

inhibits the inactivation of cyclic guanosine monophosphate

within the vascular smooth muscle cell and has the potential to

augment the effects of either endogenous or exogenously

admin-istered NO to affect vascular smooth muscle relaxation Sildenafil

can be administered in an oral or intravenous (IV) form and has

a somewhat selective pulmonary vasodilating capacity while

low-ering LA pressure and providing a modest degree of systemic

af-terload reduction in some postoperative children Chronic oral

administration of sildenafil to adults with primary pulmonary

hypertension improves exercise capacity This phenomenon has

also been demonstrated in pediatric patients with a Fontan circu-lation, perhaps suggesting a broad therapeutic application on older patients after operation for CHD

Many other vasodilators have been used with variable suc-cess in patients with pulmonary hypertensive disorders requir-ing critical care IV vasodilators—such as tolazoline, phenoxy-benzamine, nitroprusside, and isoproterenol—have little biological basis for selectivity or enhanced activity in the pul-monary vascular bed.52 However, if myocardial contractility is depressed and the afterload reducing effect on the left ventricle

is beneficial to myocardial function and cardiac output, then these drugs may be of some value In addition to drug-specific side effects, intravenous vasodilators all have the potential to produce profound systemic hypotension, critically lowering coronary perfusion pressure while simultaneously increasing intrapulmonary shunt, thus limiting their usefulness in the management of acute postoperative pulmonary hypertension

In fact, in patients with idiopathic pulmonary hypertension who have adequate LV contractility, the use of a vasopressor may help the RV coronary perfusion pressure, LV preload and systolic interventricular dependence, thus preventing a pulmo-nary hypertensive crisis

Management of Postoperative Bleeding

Infants and children undergoing cardiac surgery are at high risk for hemorrhage and need for transfusion CPB is a major throm-bogenic stimulus that causes a multifactorial coagulopathy due to dilution and consumption of clotting factors and inactivation of platelets This is further exacerbated by an immature coagulation system and the presence of hypoxia and hypothermia While upward of 79% of children undergoing cardiac surgery will re-quire transfusion,53 the need for transfusion is associated with increased morbidity.54 , 55 Transfusion criteria for packed red blood cells is determined by balancing the inherent risks associ-ated with transfusion and the need to optimize oxygen delivery

in the face of hemodynamic instability However, there are in-creasing data suggesting that the use of more restrictive transfu-sion parameters results in less transfutransfu-sion with no difference in clinical outcome.56 To minimize the need for excessive blood products, careful attention to ongoing bleeding and coagulopa-thy is necessary Baseline complete blood count and coagulation profile should be measured on return from the operating room Chest tube outputs of 10 mL/kg in the first hour or 5 mL/kg per hour for subsequent hours should prompt aggressive repletion of abnormal clotting factors, initially with platelets and fresh-frozen plasma (FFP), assessment of adequate reversal of anticoagulants, and a discussion with the surgeon When bleeding is resistant to therapy, transfusion of factor concentrates should be considered These include fibrinogen concentrate, prothrombin complex concentrates, or even activated factor VII in selected patients Significant repletion of red blood cells also necessitates the con-current transfusion of platelets and FFP to avoid further dilution

of clotting components Ongoing chest tube output that does not abate despite normalization of factors suggests the possibility

of surgical bleeding that could necessitate reexploration Further, while control of postoperative bleeding is the goal, the abrupt cessation of chest tube output—particularly when accompanied

by increasing CVP, tachycardia, and hypotension—suggests evolving tamponade Ensuring chest tube patency may be suffi-cient to reverse the process If not, reexploration of the mediasti-num may be necessary

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