Page 774 - Cardiac Nursing

750 P AR T IV / Pathophysiology and Management of Heart Disease

circulation by way of the VSD. In most cases, pulmonary blood reported 24,70,71 , prognosis does appear to be dependent on the
flow is restricted due to subvalvular or valvular pulmonary steno- age of diagnosis. When diagnosis is made during adulthood, the
sis. As a result, these patients are hypoxic and cyanosed. estimated 10-year survival rate of patients is 58%. 72 Predictors
Patients with a large VSD and little or no pulmonary restriction of mortality include functional class, heart failure, history of ar-
will have excessive pulmonary blood flow with pulmonary artery rhythmias, early age at presentation, QRS duration, and QTc
hypertension. The overload caused by the increased pulmonary interval. 66,67 Survival is worse in patients with complex lesions.
blood flow often leads to congestive heart failure. Sudden death, presumably from arrhythmias, is the usual cause
of death. Other causes of death include pulmonary infarction
Clinical Manifestations from arterial thrombosis and complications of cerebral ab-
scesses and stroke. 50 The chronic hypoxemia associated with
Clinically, the patient with tricuspid atresia is characterized by
Eisenmenger reaction results in erythrocytosis, an adaptive in-
cyanosis, clubbing of the extremities, normal or reduced pul-
crease in red blood cell (.45%) production that is due to in-
monary blood flow, a dominant left ventricle impulse, and a no-
creased erythropoietin production. Because of the viscous ef-
ticeably absent right ventricle impulse. The dominant left ventricle
fects of excessive red cell volume, a minor increase in
impulse occurs as a result of its handling both systemic and pul-
hematocrit above 65% to 75% may produce marked increase in
monary circulations, despite a decreased pulmonary blood flow.
whole blood viscosity which can lead to a number of symptoms
The physical features are dependent upon the anatomic findings.
including headache, light-headedness, myalgias, and visual dis-
Because of the absence of the tricuspid valve, the first heart sound
turbances. Therapeutic phlebotomy is rarely required in stable
is single. The presence or absence of a systolic murmur depends on
“compensated” patients, but may be indicated in symptomatic
the size of the VSD. If significant, a holosystolic murmur may be
patients with unstable hematocrits of 65% to 70%. When in-
heard at the mid to lower left sternal border and can generate a pre-
dicated, phlebotomy must be accompanied by crystalline or
cordial thrill. If the size of the VSD decreases, the murmur may
plasma exchange. 73
change from holosystolic to early systolic.
While traditional medical management has had little to offer
Management these patients, recent attention has been given to the use of selec-
tive pulmonary vasodilators in improving hemodynamics and ex-
Survival beyond infancy without surgical intervention is rare.
ercise capacity in patients with pulmonary hypertension. 74 A
However, the palliative shunts such as Blalock and Taussig (sub-
number of approved and investigation therapies are now available,
clavian artery to pulmonary artery) performed in infancy and the
offering for the first time the potential for improved survival for
increasing success of the surgical procedures such as the Fontan,
these patients.
lateral tunnel and bidirectional Glenn have contributed to an in-
creasing number of patients reaching adulthood. Introduced in
1971, the Fontan procedure has undergone a number of modi- REFERENCES
fications. However, each provides an aorticopulmonary or atrio-
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