|Year : 2007 | Volume
| Issue : 1 | Page : 53-56
Management of a case of twin pregnancy with anaemia with arteriovenous malformation in impending cardiac failure for emergency caesarean section
Chitra Juwarkar1, Lucia Misquita2, Gayatri Chandrashekar Shetty3
1 M.D., Asst. Prof, Department of Anaesthesiology, GoaMedicalCollege, Bambolim, Goa, India
2 M.B.B.S., Junior Resident, Department of Anaesthesiology, GoaMedicalCollege, Bambolim, Goa, India
3 M.D., Senior Resident, Department of Anaesthesiology, GoaMedicalCollege, Bambolim, Goa, India
|Date of Acceptance||16-Dec-2006|
|Date of Web Publication||20-Mar-2010|
18/UG2, Kamat Kinara, Miramar, Post Caranzalem, Goa. 403002
Source of Support: None, Conflict of Interest: None
A 28year old primigravida at 31.2 weeks of gestation with twin pregnancy with giant arterio-venous malformation ( AVM ) in impending cardiac failure presented for caesarean section. Diagnosis was confirmed by CT scan. The pregnancy had to be terminated at 31.2 weeks as patient's cardiac condition worsened. Here we present the anaesthetic management of this case for caesarean section
Keywords: Arteriovenous malformation, Pregnancy, Cardiac failure
|How to cite this article:|
Juwarkar C, Misquita L, Shetty GC. Management of a case of twin pregnancy with anaemia with arteriovenous malformation in impending cardiac failure for emergency caesarean section. Indian J Anaesth 2007;51:53-6
|How to cite this URL:|
Juwarkar C, Misquita L, Shetty GC. Management of a case of twin pregnancy with anaemia with arteriovenous malformation in impending cardiac failure for emergency caesarean section. Indian J Anaesth [serial online] 2007 [cited 2020 Apr 2];51:53-6. Available from: http://www.ijaweb.org/text.asp?2007/51/1/53/61117
| Introduction|| |
Congenital vascular malformations occur in approximately 1% of births with many of these not presenting for treatment. This rarity combined with severity and clinical presentation, makes clinical diagnosis difficult.  Pregnancy appears to increase the risk of bleeding from AVM. Maternal mortality associated with untreated AVM is reported to be 33%.  Pregnancy can have marked adverse effects on vascular malformations which can result in serious complications. Here we report the anaesthetic management of an unusual case of a primigravida with twin pregnancy with a giant arteriovenous malformation in impending cardiac failure for emergency Caesarean section.
| Case report|| |
A 28 year old, 48kg primigravida with 5 months amenorrhea presented to our obstetrics department with breathlessness, cough with mucoid expectoration and palpitations since 2 weeks. There was no past history of fever, chest pain or swelling of feet. On examination she was found to have pallor, tachycardia with a radial pulse of 110 beats per minute. Blood pressure was 140/70 mmHg in upper arm. On auscultation of cardiovascular system there was tachycardia with normal heart sounds and ejection systolic murmur radiating all over the chest. The initial findings were suggestive of an underlying cardiac abnormality, although she did not give a previous history of one. Echocardiography did not show any structural cardiac abnormality. Left Ventricular Ejection Fraction was 88% suggestive of hyper dynamic circulatory state. Endocrinology work up for thyroid disorder did not reveal any abnormality. Abdominal ultrasonography confirmed twin pregnancy with diamniotic dichorionic twins. Hemoglobin was 5.7gm dl -1 .
Patient was advised rest, avoidance of exertion, nutritious diet along with iron and folic acid supplementation. She was started on oral spironolactone 25 mg once daily. After a month she came back with history of breathlessness which worsened in supine position and a swelling on the back gradually increasing in size. She was found to have pallor, tachycardia, raised Jugular venous pressure, wide pulse pressure and edema of feet. Uterus was palpable at 26 weeks. Computed tomography of the thorax and upper abdomen showed a complex plexus of vascular channels along the posterolateral chest and abdominal wall extending up to mid abdomen. Few vessels were also noted on left anterolateral chest and abdominal wall. Two paired linear vascular channels comprising of an artery and vein were noted arising from subclavian vasculature. The azygous and hemiazygous venous channels were dilated. These findings confirmed a large arterio-venous malformation over left scapular region extending up to left iliac fossa. She was admitted for further obstetric management. Fetal growth was assessed by serial ultrasonography and sequential biophysical profile scores. She was started on oral digoxin 0.25mg daily five times a week, furosemide 40mg daily and spironolactone gradually increased to 40 mg twice daily. As pregnancy progressed the patient's cardiac condition started worsening. At 31.2 weeks decision was taken to terminate pregnancy by emergency caesarean section. At this time she was referred to us for administration of anaesthesia.
On preanesthetic evaluation she was breathless, unable to lie down, sitting propped up with oxygen via polymask. She was coughing continuously with mucoid expectoration.
Respiratory rate was 34/minute. On examination she had pallor, bounding pulse 90/min, blood pressure 136/70 mmHg. On Auscultation basal rales and an ejection systolic murmur were present. Abdomen was over distended at 36 weeks. There was pedal, abdominal wall and sacral edema. There was a huge swelling 30x20cms extending from the lower border of left scapula to left iliac region with dilated vessels over the surface. There was bruit over the swelling. Her haemoglobin was 7.8 gmdl -1 . ECG showed sinus tachycardia and enlarged cardiac size on chest x-ray.
| Anaesthesia management|| |
Patient was classified as ASA Grade IVE. After obtaining high risk consent, patient was shifted to operation theatre in propped up position breathing oxygen by polymask. Continuous monitoring of pulse rate, electrocardiography, oxygen saturation, and noninvasive blood pressure was carried throughout the surgery. Patient was given I.V. glycopyrrolate 0.2mg, metoclopramide 10 mg, furosemide 20mg prior to induction. Patient was preoxygenated with 100% oxygen for 5 minutes. Anaesthesia was induced with sleeping dose of thiopentone sodium (1.25%) I.V slowly. Rapid sequence intubation was carried with I.V succinylcholine 80 mg and I.V lignocaine to attenuate pressor response. Due to presence of edema of vocal cords, trachea had to be intubated with no 6.5 oral, cuffed tracheal tube. Lungs were ventilated with 100% oxygen till birth of twins. After birth of second twin, 5 units oxytocin were added to the Ringer lactate infusion. Anaesthesia was maintained with O 2 +N 2 O 50:50, I.V vecuronium 0.08 mgkg and I.V pethidine 30 mg and 20 mg which were repeated at the end of surgery. Intra operatively she was hemodynmically stable with pulse rate 110-120/min BP 130/70mmHg and SpO 2 97-99%.When auscultated at the end of surgery the basal rales had disappeared and she was hemodynamically stable. Postoperatively patient was transferred to ICU for elective ventilation and gradually weaned over 6 hours as all the parameters remained stable. Over the next few days the arteriovenous malformation gradually decreased in size. She was referred to a super specialty hospital for further management.
| Discussion|| |
According to Hamburg clinicopathological classification congenital vascular malformations (CVM) are divided into predominantly arteriovenous, venous, arterial, lymphatic, and mixed defects with each of these divided into truncular (involving main or axial vessels) or extratruncular (involving peripheral vessels) 
A.V.M are documented by the clinical staging system of Schobinger: 
Stage 1: Blush/ Stain, warmth and A.V shunting by continuous Doppler.
Stage 2: Same as stage 1 plus enlargement, tortuous veins, pulsations, thrill and bruit.
Stage 3: Same as above plus dystrophic changes, ulceration, bleeding, persistent pain or destruction.
Stage4 : Same as stage 2 plus cardiac failure.
Clinical diagnosis is confirmed by ultrasonography and doppler examination. MRI has supplanted computed tomography as it provides better soft tissue definition and avoids need of intravenous contrast agent .An arteriogram is necessary in identifying appropriate cases for surgical treatment.  Localized AVM can be excised. Isolated embolotherapy may be considered for high risk patients and critical locations where surgery would be difficult or disfiguring. Multiple, extensive, high flow macro fistulae are associated with worst prognosis and are most difficult to treat effectively.  Clinical features depend on location and size of fistula. Frequently a pulsatile mass is palpable with a thrill and bruit during systole and diastole. In case of long standing fistula there may be manifestations of chronic venous insufficiency including peripheral edema, large tortuous veins and stasis pigmentation. Skin temperature is higher over AVM.  The physiological effect relates to the combination of an uncontrolled leak from high pressure arterial system, enhanced venous return and venous pressure, resulting in increase in pulse rare and cardiac output. The pulse pressure is higher if there is a large and persistent shunt, later high output cardiac failure may occur.  In patients with high cardiac output state such as those associated with AVM, the arterial-mixed venous O 2 difference is normal or low. The mixed venous saturation is raised because of admixture of blood that is diverted away from metabolizing tissues In this condition the heart is called in to pump abnormally large quantities of blood in order to deliver O 2 required. 
Physiological changes in pregnancy : During normal pregnancy the total blood volume increases by 45%, plasma and RBC volume increase by 50% and 30% respectively resulting in physiological anaemia. The cardiac output increases by 45-50% and systemic vascular resistance decreases by 35%.CVP and PCWP are unchanged in pregnancy and left ventricular function is not hyperdynamic.  In case of twin pregnancy the physiological changes imposed on mother are in excess of those seen in singleton pregnancy. Plasma volume is increased by addition of 500 ml however there is no corresponding increase in RBC volume, resulting in exaggerated haemodilution and anemia.  Due to pressure exerted by gravid uterus on the common iliac veins the femoral venous pressure is increased.  Distensibility of veins is also increased due to progesterone according to McClausland et al.  In supine position aortocaval compression leads to decrease in venous return, cardiac output, and uterine blood flow. Patient develops compensatory mechanisms including increase in sympathetic tone and collateral routes (para vertebral to azygous vein) to improve venous return during obstruction to venacava.  During labor, cardiac output is increased by 10-25% and B.P by 5-20% with each contraction. Maternal catecholamines increased due to pain and apprehension add to cardiac work.  Additional stresses are imposed by uterine contractions which causes auto transfusion. After delivery, auto transfusion, in addition to increase central blood volume as obstruction of venacava is relieved, results in marked increase up to 80% prelobour values of stroke volume and cardiac output immediately postpartum. Patients with limited cardiac reserve may experience cardiac failure during this time.  Treatment of acute congestive cardiac failure during pregnancy aims in reducing cardiac work and bed rest, decreasing preload with diuretics, improving cardiac contractility with digitalis and other agents, reducing afterload with vasodialators. 
In this case the patient had an undiagnosed swelling on the back which gradually increased in size. It is known that in pregnancy due to hormonal effects, distensibility of veins is increased. Due to aortocaval compression alternate compensatory mechanisms like paravertebral and azygous veins become prominent to increase venous return. All this resulted in increased vascularity of AVM which acted as a shunt from high pressure to low pressure system resulting in increased pulse rate and cardiac output. In this condition the heart had to pump abnormal large quantities of blood to deliver O 2 to metabolizing tissues. As pregnancy continued, dyspnea worsened to Grade IV and basal rales were heard on auscultation. Though digoxin was started patient's condition deteriorated as pregnancy continued, hence termination of pregnancy was considered at 31.2 weeks. Normal labor increases venous return which could have caused rupture of the AVM. The increased catecholamine level, increasing cardiac work and auto transfusion, would worsen her condition so vaginal delivery was not indicated. To avoid further compromise, emergency cesarean section was planned. Regional anesthesia was contraindicated as the AVM was extending across the back. Increased vascularity would increase the chances of intravascular injection of the drug. Although regional anesthesia would have decreased the systemic vascular resistance secondary to peripheral nervous system blockade and would have decreased the cardiac output, nevertheless decrease in SVR is not predictable and not easy to control therefore regional anesthesia should probably not be selected over G.A. 
Our Goals of management in this case were:
Positioning the patient was a challenge as dyspnea worsened in the supine position. Compression of the AVM was prevented with good padding. Anaesthesia was induced with the patient tilted 30-45 o to the right. To attenuate pressor response I.V lidocaine 1.5 mgkg -1 was given prior to laryngoscopy. Engorged vasculature and edema of larynx prevented use of a larger tracheal tube. Premedication with I.V furosemide can relieve peripheral and pulmonary congestion. Although succinylcholine can abruptly increase the parasympathetic nervous system activity and could theoretically have additive effects with digitalis, we preferred succinylcholine because of our clinical experience. Anesthesia was maintained with positive pressure ventilation as it decreases the preload thereby improving the cardiac function. Vecuronium was preferred due to better cardiac stability. Low dose of opiods were given for analgesia. Intraoperatively she was infused with 300 ml Ringers lactate with 5 units oxytocin to maintain normal CVP.
- Maintain hemodynamic stability and cardiac output.
- Prevent tachycardia.
- Prevent pulmonary congestion.
- Avoid further increase in venous return and venous pressure.
Postoperatively lungs were mechanically ventilated with IPPV for 6 hours to:
- Decrease work of breathing.
- Improve oxygenation and lung volume.
- To avoid atelectasis, pulmonary edema and congestion as it redistributes water from intra alveolar to extra alveolar space.
| Conclusion|| |
Thus to conclude, pregnancy does influence both the development and progression of AVM. Fortunately these effects are uncommon and very rarely as severe as they were in the patient presented. The peripartum management of this patient was conducted successfully using a multidisciplinary approach of expertise of anesthesiology, obstetrics and medicine.[Figure 1],[Figure 2],[Figure 3],[Figure 4],[Figure 5],[Figure 6]
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]