Patient with Hypertrophic Cardiomyopathy and Retrosternal Goiter Posted for Noncardiac Surgery: Anesthesia Challenges

Anju Paul¹ https://orcid.org/0000-0001-9636-6709, Surabhi Salgaonkar², Madhuri Patil³

^1,3Department of Anesthesiology, Government Medical College Jalgaon, Maharashtra, India

²Department of Anesthesiology, Arihant Hospital, Belgaum, Karnataka, India

Corresponding Author: Anju Paul, Department of Anesthesiology, Government Medical College Jalgaon, Maharashtra, India, Phone: +91 9496673515, e-mail: anjupaul2@gmail.com

Received: 07 December 2023; Accepted: 12 July 2024; Published on: 18 December 2024

ABSTRACT

Patients with hypertrophic cardiomyopathy (HCM) pose a considerable anesthetic challenge as the outflow tract obstruction can be worsened by various conditions, which may be worsened by the concurrent occurrence of a difficult airway such as retrosternal goiter. Additionally, these patients pose a higher incidence of ischemic heart disease, which may further increase the risk of anesthesia and surgery. Successful anesthetic management in patients with HCM and goiter involves a multidisciplinary approach with the goal of maintaining stable hemodynamics with minimal LVOT obstruction, securing a definitive airway, and preventing postoperative complications. Here, we successfully managed a patient with HCM and retrosternal goiter posted for noncardiac surgery.

Keywords: Anesthesia management, Case report, Hypertrophic cardiomyopathy, Retrosternal goiter

How to cite this article: Paul A, Salgaonkar S, Patil M. Patient with Hypertrophic Cardiomyopathy and Retrosternal Goiter Posted for Noncardiac Surgery: Anesthesia Challenges. Res and Innov Anesth 2024;9(2):66–68.

Source of support: Nil

Conflict of interest: None

Patient consent statement: The author(s) have obtained written informed consent from the patient for publication of the case report details and related images.

INTRODUCTION

Hypertrophic cardiomyopathy (HCM) is a genetic cardiac disorder with an autosomal dominant trait involving a mutation of a gene encoding for sarcomere or sarcomere-associated proteins.¹ More than 1,300 mutations have been identified, and this mutation can lead to exuberant left ventricular hypertrophy (LVH).² The estimated prevalence is around 1 in 500. It has a high degree of penetrance and is more prevalent in males with a 2:1 ratio. HCM is a disorder with diverse clinical manifestations and courses. The majority of patients are asymptomatic. Symptoms of HCM include dyspnea, angina, syncope, and sudden cardiac arrest due to ventricular arrhythmias.³ Patients with HCM pose a considerable anesthetic challenge because of left ventricular outflow tract (LVOT) obstruction. This can be worsened by a decrease in preload or afterload or by sympathetic stimulation, which can lead to an increase in myocardial contractility. Additionally, the higher incidence of ischemic heart disease in patients with HCM may further increase the risk of anesthesia and surgery.

Patients with multiple diseases such as HCM and retrosternal goiter pose considerable challenges to anesthesiologists and need a multidisciplinary approach. Perioperative goals are to maintain stable hemodynamics and secure a definitive airway, which may be challenging in these types of patients. Here, we have successfully managed a patient with HCM and retrosternal goiter posted for modified radical mastectomy.

CASE DESCRIPTION

A 61-year-old unmarried female, diagnosed with hypertrophic obstructive cardiomyopathy on preoperative evaluation (on tablet metoprolol 25 mg BD) with cold abscess in the neck (Koch’s etiology) and thyroid swelling with airway compression and intrathoracic extension, was scheduled for a modified radical mastectomy. There were no cardiac or thyroid compressive symptoms. She had a history of pulmonary Koch 30 years back treated with AKT (anti-Kochs treatment). The patient had a history of thyroid swelling for 20 years for which she was on Ayurvedic medications. She also had a history of cold abscess in the neck for 4 years, which was recently diagnosed as tuberculosis cold abscess, and she was started on hepatosafe antitubercular drugs. There were no similar complaints or any sudden cardiac deaths in the family.

On examination, pulse rate was 78 bpm, regular, blood pressure was 108/60 mm Hg, and a grade 3/6 systolic murmur was heard between the apex and left sternal border. There was a midline globular swelling of 5 × 5 cm present in front of the neck, which moved with deglutition and a 3 × 3 cm globular swelling on the left side of the neck in the submandibular region. Other systemic examinations were normal. Routine blood investigation showed anemia. The thyroid function test was normal. Electrocardiogram (ECG) showed sinus rhythm, borderline LVH, and biphasic T wave in V3–V4 leads.

Chest X-ray showed bilateral healed pulmonary Koch lesions.

Echocardiography showed asymmetrical septal hypertrophy, systolic anterior wall motion of mitral valve, dynamic left outflow tract obstruction with peak systolic gradient of 85 mm Hg, mild mitral regurgitation (MR), mild tricuspid regurgitation (TR), and mild pulmonary hypertension (PH) with no diastolic dysfunction and an ejection fraction of 60%. Cardiac MRI suggested features of hypertrophic obstructive cardiomyopathy. The stress ECG and 24-hour Holter monitoring were normal. Indirect laryngoscopy showed bilateral mobile vocal cords. Contrast-enhanced computed tomography (CECT) skull base to pelvis showed right bulky thyroid with a 4.9 × 5 × 5 cm thyroid colloid nodule with compression of trachea and intrathoracic extension, submandibular gland and sternocleidomastoid abscess, and postinfective sequelae in both upper lobes of the lung. Beta-blocker was continued until the morning of surgery. An informed high-risk consent was obtained. All difficult airway equipment, cardiac drugs, and external defibrillator were kept ready. In the operating room, pulse oximetry, ECG, and blood pressure monitors were attached. Two intravenous (IV) lines were secured, and Ringer’s lactate was started. Baseline heart rate and blood pressure were 65 bpm and 130/54, respectively. To prevent anxiety and stress associated with awake fiberoptic bronchoscopy, we planned to proceed with conventional IV induction. Preoxygenation was done for 3 minutes. The patient was given injection lignocaine 2% 40 mg IV and injection fentanyl 100 µg to prevent intubation response. Rapid sequence induction and intubation were performed with injection etomidate 10 mg and injection suxamethonium 100 mg IV using C-MAC with bougie-guided armor 7 mm cuffed endotracheal tube. As the airway was difficult, we chose a short-acting muscle relaxant for intubation. General anesthesia was maintained with 50% oxygen and 50% nitrous oxide, sevoflurane at minimum alveolar concentration (MAC) of 0.8, and 6 mg of vecuronium. Injection fentanyl 50 µg was repeated before the surgical incision. During the entire intraoperative period, one episode of hypotension occurred and was managed with 150 mL of crystalloid plus injection phenylephrine 40 µg. Total output was 200 mL, and input was 400 mL. Surgery lasted for 2 hours and 15 minutes, and the patient was reversed with IV glycopyrrolate 0.4 mg and neostigmine 2.5 mg (Fig. 1). The postoperative period was uneventful, and the patient was discharged on postoperative day 1.

Fig. 1: Midline thyroid swelling of 5 × 5 cm

DISCUSSION

Hypertrophic cardiomyopathy is marked by asymmetric hypertrophy of the left ventricle, dynamic LVOT obstruction due to systolic anterior motion (SAM) of the anterior valve leaflet of the mitral valve causing MR, diastolic dysfunction, myocardial ischemia, and dysrhythmias. Myocardial ischemia can be present in patients with HCM even though they do not have coronary artery disease. HCM can be defined as nondilated LVH in the absence of any other cardiac or systemic causes of LVH. In adults, left ventricular (LV) wall thickness of >15 mm by cardiac magnetic resonance imaging (MRI) or by echocardiography is indicative of HCM. HCM can be clinically classified into (1) nonobstructive: these patients have LVOT peak gradient <30 mm Hg under basal and provocative conditions; (2) basal obstructive: these patients have resting gradient >30 mm Hg; (3) labile obstructive: these patients have resting gradient <30 mm Hg; however, gradient increases to >30 mm Hg on provocation. Up to 70% of HCM patients may have LVOT obstruction, which is associated with adverse events. Events that increase outflow tract obstruction like beta-adrenergic stimulation, hypovolemia, vasodilators, and tachycardia should be avoided.⁴ One-third of all HCM patients are of the nonobstructive type and managed medically with beta-blockers, calcium channel blockers, diuretics, and angiotensin-converting enzyme inhibitors or angiotensin receptor blockers; the remaining two-thirds of HCM patients are of the obstructive type and initially treated with beta-blockers, calcium channel blockers like verapamil, and disopyramide. Patients with an LVOT gradient above 50 mm Hg at rest or by provocation, persistent symptoms of dyspnea and chest pain [NYHA (New York Heart Association) class III or IV], and/or syncope are referred for AN invasive strategy.

Patients with tracheal compression due to goiter pose a unique challenge regarding airway management to anesthesiologists. There are different methods of induction in retrosternal goiter, which are mentioned in the literature. Conventional IV anesthesia induction, inhalational anesthesia induction, and awake fiberoptic bronchoscopy-guided tracheal intubation (oral or intranasal) are the most commonly used methods in patients with retrosternal goiter.⁵ IV anesthesia induction protocol is not contraindicated in patients with retrosternal goiter. In specific patients, especially those who cannot tolerate hypoxia or awake fiber optic bronchoscope-assisted endotracheal intubation protocols, IV anesthesia induction protocol can be used. The goal of anesthesia management includes the maintenance of preload and afterload to maintain cardiac output, avoid tachycardia, secure the definitive airway, provide lung-protective ventilation, and avoid postoperative complications. The patient was well explained about the procedure to reduce preoperative anxiety. External cardiac defibrillator and all difficult airway equipment were kept ready. Injection fentanyl and lignocaine were given through an IV route to reduce the stress response. The use of IV induction agents such as etomidate can prevent sudden decreases in systemic vascular resistance. Administration of a volatile anesthetic agent before laryngoscopy can blunt the sympathetic response. Intubation was done with a C-MAC videolaryngoscope after giving an injection of suxamethonium as we anticipated a difficult airway, and for maintenance, vecuronium was used as it decreases heart rate and thus avoids LVOT obstruction. Armor-cuffed endotracheal tubes are good options for these types of cases as they can open and freely pass the tracheal stenosis without affecting positive pressure ventilation even if the trachea is severely compromised because of their flexibility and mobility. Sevoflurane with MAC of 0.8 was used for maintenance as it may produce only mild cardiac depression and have less effect on preload and afterload. Injection fentanyl was repeated before the skin incision to reduce sympathetic response. Hypotension episodes are to be treated with volume replacement and alpha agonists such as phenylephrine.⁶ Drugs such as ephedrine and dopamine, which have beta-agonistic activity, may be avoided to prevent the worsening of LVOT obstruction.⁷ No rhythm abnormalities occurred in our case. An atrial kick is needed for adequate ventricular filling maintenance. So, maintenance of sinus rhythm is important. Intraoperative supraventricular tachycardia can be managed by pharmacological or electrical cardioversion. Beta-blocker and amiodarone can be useful in controlling the arrhythmias.

Clinical Significance

Patients with HCM and Goiter need a multidisciplinary approach. Perioperative goals are to maintain stable hemodynamics by maintaining preload and afterload, preventing dynamic LVOT obstruction, and securing a definitive airway.

ORCID

Anju Paul https://orcid.org/0000-0001-9636-6709

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