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Table of Contents
Year : 2011  |  Volume : 14  |  Issue : 2  |  Page : 115-118
Anesthetic management for combined mitral valve replacement and aortic valve repair in a patient with osteogenesis imperfecta

1 Department of Anesthesia, Jewish Hospital and St. Mary's Healthcare, Louisvile KY; Department of Anesthesiology and Perioperative Medicine, University of Louisville, Louisvile KY, USA
2 Department of Anesthesiology and Perioperative Medicine, University of Louisville, Louisvile KY, USA

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Date of Submission14-Aug-2010
Date of Acceptance25-Oct-2010
Date of Web Publication25-May-2011


Osteogenesis imperfecta is a rare disorder of connective tissues and presents multiple challenges, including difficult airway, hyperthermia, coagulopathy and respiratory dysfunction, for anesthesiologists, especially during cardiac surgery. We present anesthetic management of a patient with osteogenesis impertecta during double valve surgery. Dexmedetomidine infusion minimized the risks of malignant hyperthermia. Glidescope and in-line stabilization facilitated endotracheal intubation and protected his oral structures and cervical spine. Transesophageal echocardiography (TEE) diagnosed a flail A3 segment and redundant left coronary cusp causing mitral and aortic regurgitation. The mitral valve was replaced and the aortic valve repaired. Coagulopathy was corrected according to comprehensive coagulation analysis. Glidescope, dexmedetomidine, coagulation analysis and TEE could facilitate anesthetic management in these patients.

Keywords: Aortic valve repair, mitral valve replacement, osteogenesis imperfecta

How to cite this article:
Huang J, Dinh M, Kuchle N, Zhou J. Anesthetic management for combined mitral valve replacement and aortic valve repair in a patient with osteogenesis imperfecta. Ann Card Anaesth 2011;14:115-8

How to cite this URL:
Huang J, Dinh M, Kuchle N, Zhou J. Anesthetic management for combined mitral valve replacement and aortic valve repair in a patient with osteogenesis imperfecta. Ann Card Anaesth [serial online] 2011 [cited 2020 Sep 28];14:115-8. Available from:

   Introduction Top

Osteogenesis imperfecta (OI) is a rare genetically inherited connective tissue disorder with characteristic blue sclera, brittle bones, hearing loss, dental abnormalities and cardiac involvements. Fewer than 40 cases of valvular surgery in OI patients have been reported with a high mortality rate. [1] These cases present multiple challenges for anesthesiologists, such as airway control, hemostasis, possibly increased risk of malignant hyperthermia, respiratory dysfunction from deformed thoracic cavity and pathological changes of heart valves. [2] The authors have describe here novel anesthetic innovations for managing this patient with OI who required combined mitral valve replacement and the aortic valve repair

   Case Report Top

A 20-year-old male with worsening heart failure symptoms presented for mitral and aortic valve repair or replacement. His medical history was significant for OI type I, right eye blindness, asthma, scoliosis, cardiomyopathy and pulmonary hypertension. He had blue left eye sclera, short stature, scoliosis of the spine and a loud systolic and diastolic murmur heard along the left sternal border. He weighed only 40 kg. Twelve-lead ECG showed sinus tachycardia with right ventricular hypertrophy. Echocardiography showed ejection fraction of 40%, severe mitral regurgitation and moderate to severe aortic regurgitation. Airway examination revealed a Mallampati II oropharygeal view, full movement of the neck and thyroidmental distance more than 6 cm. Coagulation studies were normal with international normalized ratio 1.30, partial thromboplastin time 29 seconds, platelet counts 214,000/mm 3 , and platelet aggregation 96% (normal 80-97%) by Platelet Works (Helena Laboratories, Beaumont, TX, USA). Analysis with Sonoclot (Sienco Inc., Arvada, CO, USA) showed normal activated coagulation time 141 seconds (normal 119-195 seconds), clot rate 21 U/min (normal 7-32 U/min) and platelet function 3.9 (normal 1.0-17.0).

To avoid possible occurrence of malignant hyperthermia, the anesthesia machine was flushed with high flow oxygen for 30 min and all vaporizers were removed from both the anesthesia and heart-lung machines. The control knob of nitrous oxide was taped and marked. Caution was exercised during positioning and transferring to avoid any excessive force so as to prevent the possibility of fracture of his delicate bones. No blood pressure cuff was applied due to concerns for the same reason. A radial arterial line was placed and all pressure points were carefully padded with foams and pillows. End tidal CO 2 , and nasal and bladder temperature were monitored. The relatively normal airway examination and the patient's extreme anxiety promoted us to avoid an awake fiberoptic intubation. The difficult airway cart with different sizes of laryngeal mask airway (LMA) was ready in the operation theater. The plan was to use Glidescope (Verathon, Inc., Bothell, WA, USA) for intubation after induction. If the intubation was difficult, an LMA would be inserted and a fiberoptic intubation through LMA would be attempted. General anesthesia was induced with fentanyl and etomidate (Hospira, Lake Forest, IL, USA). Succinylcholine was avoided due to concerns for possible initiation of malignant hyperthermia and fasciculation induced fracture. After easy mask ventilation was established, rocuronium was administered to facilitate intubation. An adult Glidescope was inserted into the mouth with extra caution to avoid contact with teeth and maxilla. While an assistant maintained the neck in a neutral position, grade II view was obtained with minimal force on the Glidescope and a styletted 7 size endotracheal tube was passed into the trachea without difficulty.

Anesthesia, analgesia, and muscle relaxation were achieved with dexmedetomidine 0.6 mcg/kg/hour and propofol 50 mcg/kg/min infusions and intermittent doses of fentanyl and rocuronium. Inhalational agents were avoided due to the possibility of occurrence of malignant hyperthermia and dexmedetomidine was used to provide both sedation and analgesia. Depth of anesthesia was monitored with bispectral index (BIS) monitoring (Aspect, Norwood, MA, USA) and BIS was kept below 50 during the procedure Cerebral oxygenation was monitored with INVOS 5100 (Somanetics Corporation, Troy, MI, USA) and maintained above 40. Right internal jugular central line and a pulmonary artery catheter were placed without difficulty, with the head on a pillow and maintained in a neutral position. Pulmonary artery pressure was 70/30 mmHg. Intraoperative transesophageal echocardiography (TEE) demonstrated dilated left ventricle with an ejection fraction of 40%. A newly diagnosed flail A3 segment from midesophageal four-chamber and commissural views and severely dilated mitral annulus contributed to severe mitral regurgitation. The left coronary cusp was found to be redundant and prolapsing causing moderate aortic regurgitation [Figure 1]a and b,[Figure 2]a, and b. The geometry of aortic root and aorta was normal in size.
Figure 1: (a)Midesophageal aortic valve short axis view showing prolapsed left coronary cusp (LCC = left coronary cusp)
Figure 1b: Color flow Doppler showing moderate aortic regurgitation at the right and left coronary cusp junction (AR = aortic regurgitation, LCC = left coronary cusp, RCC = right coronary cusp)

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Figure 2: (a)Midesophageal long axis view showing prolapsed left coronary cusp (LCC = left coronary cusp)
Figure 2b: Color flow Doppler showing eccentric aortic regurgitation at the right and left coronary cusp junction (AR = aortic regurgitation)

Click here to view

As per the policy in our institution, 5 g aminocaproic acid was administered prior to, on and after cardiopulmonary bypass (CPB) to inhibit fibrinolysis. Heparin was used to achieve anticoagulation for CPB. The patient was cooled to 31.8°C. Surgeon confirmed our echocardiographic findings and elected to replace the mitral valve with a 23-mm St. Jude mechanical valve prosthesis (St. Jude Medical, St. Paul, MN, USA). The aortic valve was repaired by plication and commissural pledgetted sutures. Postoperative TEE confirmed that mitral prosthesis was well seated without para-valvular leak and there was only trace of residual aortic regurgitation.

Total bypass time was 167 min including 124 min aortic cross-clamping time. The patient came off CPB on 40 ppm inhaled nitric oxide in order to achieve the baseline pulmonary artery pressure. Milrinone (0.5 mcg/kg/min), epinephrine (0.1 mcg/kg/min) and dobutamine (10 mcg/kg/min) intravenous infusions were also initiated. Norepinephrine and vasopressin infusions were used to counter the low systemic vascular resistance after CPB. His cardiac output was 4.1 l/min, blood pressure 115/65 mmHg and pulmonary artery pressure 63/31 mmHg. Postbypass coagulation studies indicated international normalized ratio 2.12, partial thromboplastin time 38.2 seconds, fibrinogen level 212 mg/dl (normal 224-512 mg/dl), platelet count 103,000/mm 3 and platelet aggregation of 89% by PlateletWorks. Sonoclot demonstrated activated coagulation time of 216 seconds, clot rate 12.2 U/min and platelet function of 4.1. With the coagulation panel being in the low normal range and active bleeding from the surgical filed, which continuously depleted platelets and coagulation factors, it was decided to transfuse one unit of packed red blood cell, 440 ml of cell saver blood, 4 units of fresh frozen plasma, 10 units of cryoprecipitate, and 1 multi-donor pharesis unit of platelet to correct the coagulopathy. During the entire procedure his temperature was within the range of 31.8-36.8°C. His temperature was continuously monitored for 48 hours postoperatively to rule out possible malignant hyperthermia. The patient was slowly weaned off the nitric oxide and received a tracheotomy due to requirement of prolonged postoperative ventilation on postoperative day 10. Unfortunately, he was found fallen out of the bed by himself despite full fall precautions and detached from the ventilator on postoperative day 15 and died. Emergent TEE during resuscitation demonstrated well functioning mitral and aortic valves.

   Discussion Top

Four types of OI have been described. The severity of the disease depends on the site of mutation and resultant quantitative or qualitative deficits in the collagen macromolecule. Type I is the most common and mild type characterized by blue sclera and variable bone fragility. Type II manifests as early as in utero or at birth and type III patients usually die within the second to fourth decades of life, complicated with vertebral compression fractures and progressive pulmonary involvement. Type IV OI is similar to type I, with the exception of the classic blue sclera feature. More recently, other forms have been identified. [1]

Airway control issues in patients with OI include possible difficult airway, increased risk for odonto-axial dislocation, brittle teeth, and weakened cervical and mandibular bones which could be easily fractured during laryngoscopy. [3] Successful intubations using fiberoptic bronchoscope or LMA have been reported. [4] We utilized Glidescope and in-line stabilization to minimize cervical motion and provide better views for possible anteriorly positioned vocal cord. From our experiences, Glidescope could provide better airway views with minimal force on teeth, maxilla and cervical spines to achieve successful intubation in OI patients.

The mechanism of perioperative hyperthermia in OI patients has been linked to either malignant hyperthermia or increased thyroid hormones. Total intravenous anesthesia has been highly recommended. [5] Succinylcholine should be avoided due to possible malignant hyperthermia as well as muscle fasciculation which might injure those fragile bones. We used dexmedetomidine to provide both amnesia and analgesia from its alpha two agonist property intraoperatively. Our case showed that dexmedetomidine could be a very useful adjunct in OI patients' perioperative anesthetic management.

Patients with OI are less likely to have cardiovascular involvement compared to those with Marfan and Erlers-Danlos syndromes. [1] Aortic regurgitation is the most common valvular abnormality in OI, caused by bicuspid aortic valve, dilatation of the aortic root, aneurysm of the sinus of Valsalva, thin and elongated aortic cusps, cystic medial mucinous or myxoid degeneration and disorganized collagen architecture. Mitral valve regurgitation occurs even lesser, with leaflets redundancy and annular dilatation being the main etiologies. Only nine cases of combined mitral and aortic valve repair/replacement have been reported in the literature. [1] Intraoperative TEE provides invaluable information regarding anatomy and function of heart valves. With a ruptured chordae and flail A3, concerns for durability of mitral valve repair and the patient's young age had driven the decision to replace the mitral valve. TEE correctly diagnosed a redundant and prolapsing left coronary cusp causing aortic regurgitation, which facilitated the first aortic valve repair since 1960s in OI patients. Nitric oxide, as a specific pulmonary artery dilator, could have an important role in the management of reversible pulmonary hypertension caused by mitral and aortic regurgitations in OI patients.

OI patients have a higher risk of bleeding during open heart surgeries. Platelet dysfunction from defective release of platelet factor III or inability to aggregate with adenosine diphosphate has long been speculated. [1],[6] In our patient, platelet counts, not platelet function, were impaired after CPB, according to Platele Works and Sonoclot. PlateletWorks measures the rate and degree of platelets aggregation after coating the tube with adenosine diphosphate. Sonoclot provides information on the entire hemostasis process to detect the viscoelastic changes of a whole blood sample. PlateletWorks and Sonoclot evaluation in OI patients could help our understanding and correction of coagulopathy in this patient population.

In conclusion, several unique anesthetic techniques,were used to manage the case. They were, use of Glidescope, dexmedetomidine, TEE, PlateletWorks and SonoClot, for combined aortic valve repair and mitral valve replacement in a patient with OI.

   References Top

1.Bonita R, Cohen I, Berko B. Valvular heart disease in osteogenesis imperfecta: Presentation of a case and review of the literature. Echo cardiography 2010;27:69-73.  Back to cited text no. 1
2.Porsborg P, Astrup G, Bendixen D, Lund AM, Ording H. Osteogenesis imperfecta and malignant hyperthermia. Is there a relationship? Anesthesia 1996;51:863-5.  Back to cited text no. 2
3.Maya D, Nayyar B, Patra P. Anesthetic Management of a case of osteogenesis imperfecta with associated bronchial asthma for repair of corneal perforation. Indian J Anaesth 2006;50:223-5.  Back to cited text no. 3
4.Santos ML, Añez C, Fuentes A, Méndez B, Periñán R, Rull M. Airway management with ProSeal LMA in a patient with osteogenesis imperfecta. Anes Analg 2006;103:794.  Back to cited text no. 4
5.Ogawa S, Okutani R, Suehiro K. Anesthetic management using total intravenous anaesthesia with remifentanil in a child with osteogenesis imperfecta. J Anesth 2009;23:123-5.  Back to cited text no. 5
6.Bhandari G, Shahi KS, Bhadoria P, Bhalotra AR, Sandhya OD, Arya M. Osteogenesis imperfecta: No place for imperfect anaesthesiologist. Indian J Anaesth 2008;52:577.  Back to cited text no. 6
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Correspondence Address:
Jiapeng Huang
Department of Anesthesia, 200 Abraham Flexner Way, Louisville KY
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9784.81566

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