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to measure cerebral blood flow during C/S under slowly induced extensive cutaneous vascular malformations, venous varicosities,
epidural anesthesia.147 However, the value of transcranial Doppler and focal abnormalities of the deep venous system. In addition,
in this setting is uncertain. An arterial catheter is recommended for there is underlying soft-tissue and bony hypertrophy that can give
continuous BP monitoring during labor and delivery.148 rise to arm and leg asymmetry and facial asymmetry. The latter
may give rise to temporomandibular joint dysfunction.160 Other
orthopedic manifestations of KTWS include limb-length discre-
Kawasaki disease (mucocutaneous lymph
pancies, digital anomalies, ulcerations, spine and hip abnormal-
node syndrome)
ities, and Charcot osteoarthropathy.161
Kawasaki disease (KD) is an acute febrile illness of children under Klippel-Trenaunay-Weber syndrome can be diagnosed in utero
using routine prenatal ultrasound.162,163 Women with KTWS can
the age of four years. Most investigators agree that an infectious

1 Cardiovascular and respiratory disorders

become pregnant and there are a number of case reports describ-
ing KTWS and pregnancy.164,165,166,167 Klippel-Trenaunay-Weber 1. Roberts, N. V. & Keast, P. J. Pulmonary hypertension and pregnancy: a lethal
syndrome is associated with bleeding from angiomata in the combination. Anaesth. Intensive Care 1990; 18: 366“74.
genitalia and coagulation disorders. Uterine angiomatosis168 2. Cohen, R., Talwar, A. & Efferen, L. S. Exacerbation of underlying pulmonary
disease in pregnancy. Crit. Care Clin. 2004; 20: 713“30.
may lead to placental insufficiency and fetal growth restriction.169
3. Rich, S. Primary pulmonary hypertension. Prog. Cardiovasc. Dis. 1988; 31:
Kasabach-Merritt coagulopathy, defined by thrombocytopenia
and a consumptive coagulopathy, can complicate KTWS during 4. Mangano, D. T. Anesthesia for the pregnant cardiac patient. In Hughes,
pregnancy especially if there are extensive hemangiomas.170 This S. C., Levinson, G. & Rosen, M. A. (eds), Shnider and Levinson™s Anesthesia
has obvious implications for the obstetric anesthesiologist. for Obstetrics, 4th edn. Philadelphia: Lippincott Williams and Wilkins, 2001;
pp. 471“2.
Major conduction anesthesia has been described in women
5. Harnett, M., Mushlin, P. S. & Camann, W. R. Cardiovascular disease. In
with KTWS during pregnancy, but it is prudent to obtain MRI or
Chestnut, D. H. (ed.), Obstetric Anesthesia: Principles and Practice, 3rd
CT studies to rule out AV malformations of the lumbosacral edn. Philadelphia: Elsevier Mosby, 2004; pp. 710“11.
spine.171,172 Epidural needles should not be placed through a 6. Fuster, V., Steele, P. M., Edwards, W. D. et al. Primary pulmonary hyperten-
cutaneous port-wine lesion.171,173 sion: natural history and the importance of thrombosis. Circulation 1984;
70: 580“7.
7. Takeuchi, T., Nishii, O., Okamura, T. & Yaginuma, T. Primary pulmonary
Splenic artery aneurysm hypertension in pregnancy. Int. J. Gynecol. Obstet. 1988; 26: 145“50.
8. Nelson, D. M., Main, E., Crafford, W. & Ahumada, G. G. Peripartum heart
This uncommon aneurysm has the potential to grow and rupture failure due to primary pulmonary hypertension. Obstet. Gynecol. 1983; 62:
during pregnancy with life-threatening, often fatal, results for the S58“63.
mother and fetus.174 Splenic artery aneurysm (SAA) occurs pre- 9. Dawkins, K. D., Burke, C. M., Billingham, M. E. et al. Primary pulmonary
hypertension and pregnancy. Chest 1986; 89: 383“8.
dominantly in women, and a majority of the aneurysms are
10. Feijen, H. W. H., Hein, P. R., van Lakwijk-Vondrovicova, E. L. & Nijhuis,
asymptomatic until rupture. Over half of those that rupture do
G. M. Primary pulmonary hypertension and pregnancy. Eur. J. Obstet.
so during pregnancy or in women who have had children.175 Gynecol. Reprod. Biol. 1983; 15: 159“64.
A rupture of the splenic artery can mimic uterine rupture, severe 11. Weiss, B. M., Zemp, L., Seifert, B. & Hess, O. M. Outcome of pulmonary
placental abruption, ectopic pregnancy, and most acute abdom- vascular disease in pregnancy: a systematic overview from 1978 through
1996. J. Am. Coll. Cardiol. 1998; 31: 1650“7.
inal emergencies. It may also mimic cardiorespiratory arrest from
12. Naeije, R. & Vachiery, J. L. Medical therapy of pulmonary hypertension.
pulmonary embolism.176 A case of SAA at a hospital in San Diego
Conventional therapies. Clin. Chest Med. 2001; 22: 517“27.
occurred on the postpartum unit in an obese woman who com- 13. Rubin, L. J., Badesch, D. B., Barst, R. J. et al. Bosentan therapy for pulmonary
plained of chest pain and shortness of breath. During evaluation arterial hypertension. N. Engl. J. Med. 2002; 346: 896“903.
of her symptoms and treatment of hypoxemia she had a cardiac 14. O™Hare, R., McLoughlin, C., Milligan, K., McNamee, D. & Sidhu, H.
Anaesthesia for caesarean section in the presence of severe primary pul-
arrest with electromechanical dissociation (EMD) and efforts at
monary hypertension. Br. J. Anaesth. 1998; 8: 790“2.
resuscitation failed. Postmortem examination revealed a large
15. Lam, G. K., Stafford, R. E., Thorp, J., Moise, K. J., Jr & Cairns, B. A. Inhaled
hemoperitoneum and a ruptured splenic artery aneurysm. nitric oxide for primary pulmonary hypertension in pregnancy. Obstet.
Patient survival has been described when timely laparotomy is Gynecol. 2001; 98: 895“8.
performed and treatment started with rapid infusion of i.v. fluids 16. Bildirici, I. & Shumway, J. B. Intravenous and inhaled epoprostenol for
primary pulmonary hypertension during pregnancy and delivery. Obstet.
and blood products, ligation of the proximal splenic artery, and
Gynecol. 2004; 103: 1102“5.
splenectomy.177 Awareness of the possibility of splenic artery
17. Stewart, R., Tuazon, D., Olson, G. & Duarte, A. G. Pregnancy and primary
aneurysm rupture is key to timely and successful resuscitation. pulmonary hypertension: successful outcome with epoprostenol therapy.
Chest 2001; 119: 973“5.
18. Hill, L. L., De Wet, C. J., Jacobsohn, E., Leighton, B. L. & Tymkew, H.
Peripartum substitution of inhaled for intravenous prostacyclin in a
patient with primary pulmonary hypertension. Anesthesiology 2004; 100:
This chapter has dealt with uncommon vascular disorders that have
the potential to cause significant morbidity and mortality to mother
19. Breen, T. W. & Janzen, J. A. Pulmonary hypertension and cardiomyopathy:
and fetus. A more common cause of maternal mortality from a anaesthetic management for Caesarean section. Can. J. Anaesth. 1991; 38:
vascular disorder is venous thromboembolism, which has an inci- 895“9.
dence of 1 per 1000 deliveries178 of which 1“2% are fatal.179 Acquired 20. Sullivan, J. M. & Ramanathan, K. B. Management of medical problems in
pregnancy: severe cardiac disease. N. Engl. J. Med. 1985; 313: 304“9.
or inherited thrombophilias augment the risk. Arterial thrombosis
21. Braun, E. B., Palin, C. A. & Hogue, C. W. Vasopressin during spinal anesthe-
is a feature of a number of the uncommon conditions reviewed, and
sia in a patient with primary pulmonary hypertension treated with intrave-
the incidence of ischemic stroke during pregnancy is estimated at nous epoprostenol. Anesth. Analg. 2004; 99: 36“7.
0.18 per 1000 deliveries. Risk factors for arterial thrombosis include 22. Slomka, F., Salmeron, S., Zetlaoui, P. et al. Primary pulmonary hypertension
advanced maternal age, atherosclerosis, obesity, hypertension, and and pregnancy: anesthetic management for delivery. Anesthesiology 1988;
69: 959“61.
smoking. Other important risk factors include prosthetic heart
23. Nootens, M. & Rich, S. Successful management of labor and delivery in
valves and drugs that cause vasospasm.180 Obstetric anesthesio-
primary pulmonary hypertension. Am. J. Cardiol. 1993; 71: 1124“5.
logists need to be aware that women with predisposition to vascular 24. Robinson, D. E. & Leicht, C. H. Epidural analgesia with low-dose bupiva-
disease or those with preexisting vascular lesions may become caine and fentanyl for labor and delivery in a parturient with severe pul-
symptomatic during pregnancy with clinically significant results. monary hypertension. Anesthesiology 1988; 68: 285“8.

Chapter 3

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27. Khan, M. J., Bhatt, S. B. & Kryc, J. J. Anesthetic considerations for parturients angiectasia (Osler-Weber-Rendu syndrome): a view from the 21st century.
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1 Cardiovascular and respiratory disorders

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John Philip and Shiv K. Sharma

Adult respiratory distress syndrome Worsening hypoxemia impairs O2 delivery to tissues causing
multisystem organ dysfunction, typically acute renal failure, dis-
seminated intravascular coagulopathy, and hepatic failure.
Adult respiratory distress syndrome (ARDS) is a severe form of Multisystem organ failure is the main cause of death.
acute respiratory failure that can develop following a systemic or
pulmonary insult. Adult respiratory distress syndrome is not Diagnosis
unique to adults, and in children is known as ˜˜acute respiratory
The clinical spectrum of ARDS is wide. In 1994, the American“
distress syndrome™™. The incidence of ARDS in pregnancy is vari-
European consensus conference on ARDS8 issued the following
ably reported as 1 in 3000 to 1 in 6000 deliveries1 with mortality as
definition that has been widely adopted by clinicians and
high as 44%.1,2,3,4
researchers. ARDS is characterized by: (1) bilateral radiographic
pulmonary infiltrates; (2) PaO2 to FiO2 ratio of 200 or less regard-
less of the level of positive end-expiratory pressure (PEEP); (3) no
clinical evidence of heart failure (if measured, a pulmonary capil-
Several disorders can cause ARDS in pregnancy (see Table 4.1).
lary wedge pressure (PCWP) of 18 mmHg or less).
Sepsis, secondary to pyelonephritis, chorioamnionitis, or endo-
metritis, is a common cause of ARDS in pregnancy.1,2,5 Other
causes include obstetric hemorrhage, severe preeclampsia, and Medical management
aspiration.1,2,5 There may be a combination of sepsis, shock, and
The management of ARDS in pregnancy does not differ signifi-
fluid overload, the latter of which can be exacerbated by tocolytic
cantly from that in nonpregnant patients. The main objectives in
managing ARDS are to treat the underlying cause, optimize tissue
O2 delivery, and manage the acute lung injury while limiting
further lung injury.
General principles of management include provision of res-
Following the initial insult, a number of inflammatory mediators
piratory support to ensure adequate oxygenation; support of CO
such as tumor necrosis factor and interleukins 1, 6, and 8 are
with fluids and inotropes to promote O2 delivery; correction of
released. Neutrophils are activated to release other mediators
anemia to facilitate O2 delivery; and administration of sedatives,
such as reactive oxygen (O2) species and proteases. These medi-
analgesics, and antipyretics to reduce O2 consumption. Sepsis is
ators produce widespread microvascular and alveolar epithelial

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