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Laparoscopic Rectopexy in a Patient With Pulmonary Hypertension Associated With Scleroderma: A Case Report

Montandrau, Olivier MD*; Mascitti, Paola MD*; Boucau, Cécile MD*; Cosserat, Julie MD; Denet, Christine MD; Beaussier, Marc MD, PhD*; Philip, Ivan MD*

doi: 10.1213/XAA.0000000000000919
Case Reports
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We report the perioperative management of a patient with pulmonary hypertension under new-generation treatments who underwent laparoscopic surgery. Preoperatively, arterial catheter, central venous line, and transesophageal echocardiography probe were inserted in addition to standard monitoring. Intraoperatively, inhaled nitric oxide was used because of increasing pressure in the right heart chambers related to the Trendelenburg position and the pneumoperitoneum. The operation finally lasted <2 hours without complication. The prognosis of patients with pulmonary hypertension has evolved since the advent of new management strategies. Thorough preoperative assessment and multidisciplinary discussion in a referral center are essential for medical optimization.

From the Departments of *Anesthesia

Internal Medicine

Digestive Surgery, Institut Mutualiste Montsouris, Paris, France.

Accepted for publication September 4, 2018.

Funding: None.

The authors declare no conflicts of interest.

Address correspondence to Olivier Montandrau, MD, Department of Anesthesia, Institut Mutualiste Montsouris, 42 Boulevard Jourdan, 75014 Paris, France. Address e-mail to olivier.montandrau@imm.fr.

Pulmonary hypertension (PH) has long been considered as an important predictor of outcomes in patients undergoing noncardiac surgery.1 However, little is known about the prognosis of these patients after laparoscopic surgery,2,3 particularly since the emergence of new therapies such as endothelin-1 receptor antagonists or phosphodiesterase-5 inhibitors. We report the perioperative management of a patient with pulmonary arterial hypertension (PAH) who underwent laparoscopic surgery for rectal prolapse after optimization with bosentan and tadalafil. Our aim is to highlight the need of thorough preoperative evaluation and the importance of appropriate monitoring in these patients. The patient has provided written consent to publish this case report.

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CASE DESCRIPTION

A 69-year-old woman who presented with a history of scleroderma associated with PH type 1 or PAH was scheduled for laparoscopic rectopexy under general anesthesia for recurrent rectal prolapse with fecal incontinence. The procedure had to be initially cancelled after the anesthesia preoperative evaluation because of her unstable pulmonary status with tachypnea, New York Heart Association class III dyspnea, and signs of right heart failure. An initial transthoracic echocardiography (TTE) at rest showed right atrium (RA) and right ventricle (RV) enlargement with normal contractile function; pulmonary arterial systolic pressure (PASP), assessed from the peak tricuspid regurgitant (TR) velocity, was estimated at 75 mm Hg on a moderate TR. There was no left-to-right shunt, and the left ventricle had normal size and performance. Hence, she was referred to the French National Centre of Pulmonary Hypertension for further testing and optimization. Severity of PAH was assessed by the 6-minute walk test (6MWT), right heart catheterization (RHC), arterial blood gas (ABG), and pulmonary functional tests with diffusing capacity of the lung for carbon monoxide. The findings before and at 6 months after the initiation of treatment are summarized in the Table. After the referral, she was prescribed bosentan 125 mg twice daily, tadalafil 40 mg, furosemide 40 mg, and spironolactone 25 mg once daily. After a second anesthesia consultation, given the improvement of her pulmonary condition, surgery was planned for a few days later. The laparoscopic approach and monitoring were discussed before the procedure, and the patient was clearly informed about the risk of laparoconversion and the possible occurrence of serious complications.

Table.

Table.

Figure 1.

Figure 1.

Figure 2.

Figure 2.

On the day of surgery, the patient did not receive premedication except for her PAH-specific therapies. In addition to standard monitoring, an arterial catheter, a central venous line, and a transesophageal echocardiography (TEE) probe were inserted. The arterial catheter was placed before administration of any sedation. Anesthesia was induced with target-controlled infusion of propofol with a target of 3 μg·mL−1, sufentanil 0.3 ng·mL−1 (Base Primea; Fresenius Kabi, Bad Homburg, Germany), and intravenous bolus of atracurium 0.5 mg·kg−1. Mechanical ventilation was initiated to 7 mL·kg−1 tidal volume with a 70/30 mixture of oxygen/air without positive end-expiratory pressure. A right jugular venous catheter was inserted to monitor the central venous pressure (CVP), which was at 6 mm Hg before incision. Anesthesia was maintained to keep Bispectral Index values at 50 ± 10. TEE showed normal-sized RA and RV with preserved systolic function, and the estimated PASP was 35 mm Hg. ABG revealed the following results: pH 7.38 mm Hg, Pco2 37 mm Hg, Po2 250 mm Hg, bicarbonate 22 mmol·L−1, hemoglobin 11.1 g·dL−1, and an arterial to Etco2 pressure gradient of 5 mm Hg. The jugular venous oxygen saturation (Svjo2) was 74%. Intraoperatively, pneumoperitoneum was gently initiated with carbon dioxide insufflation, maintaining the abdominal pressure <12 mm Hg and the patient was placed in steep 45° Trendelenburg position. At that moment, CVP rose significantly from 6 to 16 mm Hg, Etco2 decreased from 36 to 30 mm Hg, and TEE showed a right heart dilation with a severe TR, PASP being estimated at 67 mm Hg, whereas RV systolic function, assessed by the tricuspidal annular plane systolic excursion and by visual estimates, remained stable (Figure 1). As a consequence, we reduced the Trendelenburg position and started inhaled nitric oxide (iNO) at initial dose of 10 ppm to decrease pulmonary vascular resistance (PVR) and pulmonary arterial pressure. Fifteen minutes after iNO administration, CVP and estimated PASP decreased to 7 and 35 mm Hg, respectively, the mean arterial pressure and Etco2 remained steady, and Svjo2 rose at 82% (Figure 2). When effective, iNO was decreased at 5 ppm and the patient was repositioned in Trendelenburg position without hemodynamic disorders. Surgery was uneventful and lasted for 90 minutes. The patient received 250 mL of Ringer’s solution, and no vasopressors were used. Neuromuscular blocking agent was antagonized with neostigmine 0.04 mg·kg−1 and atropine 0.015 mg·kg−1. The patient was extubated in the operating room 30 minutes after surgery and transferred to the intensive care unit as planned for postoperative monitoring. After extubation, iNO was delivered through a facial mask keeping the same dose and was gradually withdrawn 6 hours after surgery without rebound effect. Postoperative pain was minor and controlled without the use of opioids. Normal diet and medical regimen were reintroduced the day after surgery when she was shifted to the ward. She was discharged from the hospital 3 days later.

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DISCUSSION

Systemic scleroderma is an autoimmune progressive connective tissue disorder characterized by excessive collagen production and accumulation resulting in organ fibrosis, particularly in blood vessels, skin, and lungs.4 Pulmonary damage occurs in 33%–50% of cases with development of an interstitial syndrome, and in 8%–12% of patients develop PAH.5 PH is defined as pulmonary arterial mean pressure ≥25 mm Hg measured by RHC at rest.6 It is classified into 5 different categories according to the World Health Organization classification.7 The term “PAH” describes a group of PH patients characterized by the presence of precapillary PH, defined by a pulmonary arterial wedge pressure <15 mm Hg and a PVR >3 Wood units in the absence of other causes of precapillary PH, caused by changes in lung microcirculation. The evolution is characterized by a gradual increase in PVR which can lead to right heart failure. Specific therapies target 3 separate pathways involved in PAH: endothelin-1 receptor antagonists (bosentan, macitentan, and ambrisentan), phosphodiesterase-5 inhibitors (sildenafil and tadalafil), or prostacyclin analogs (epoprostenol, treprostinil, and iloprost). The survival rate has considerably improved since the emergence of these therapies. However, the perioperative management of these complex patients remains challenging, the perioperative prognosis being classically poor.8 It requires a multidisciplinary approach with anesthesiologists, PH experts, and surgeons. A thorough preoperative physical examination may reveal dyspnea, fatigue, edema, or palpitations, but these signs are not specific and may not be noticeable in early stages. Further tests should include 6MWT, RHC, TTE, and pulmonary function testing with diffusing capacity of the lung for carbon monoxide to graduate the severity of the disease and to optimize the treatment.9 Several risk factors are associated with increased morbidity and mortality: New York Heart Association class ≥2, 6MWT <300 m, history of coronary disease, pulmonary embolism or chronic renal insufficiency, emergency surgery, American Society of Anesthesiologists physical status >II, duration of anesthesia >33 hours, intraoperative use of vasopressors, and right ventricular hypertrophy with severe systolic dysfunction.10 One of these factors should prompt reevaluation of the need for surgery, particularly in the presence of significant RV failure.

The main principles of the anesthetic management are the prevention of systemic hypotension (risk of RV ischemia), the prevention of acute elevation in PVR and RV afterload (risk of RV failure), and the maintenance of RV preload and systolic function. Thus, systemic vasodilatation, hypo- or hypervolemia, and factors known to exacerbate pulmonary vasoconstriction (hypoxia, hypercapnia, acidosis, hypothermia, and uncontrolled pain) must be avoided. Consequently, it is essential to elaborate a plan taking into account these parameters: the use of an arterial catheter is necessary because hemodynamic changes can occur rapidly and it allows iterative ABG sampling to check adequacy of ventilation. Central venous catheter provides continuous RA pressure measurement, which reflects the relationship of blood volume to the capacity of the venous system, the functional capacity of the RV, and the detection of new onset or worsening of TR. Furthermore, it allows intermittent sampling of Svjo2 and administration of vasopressors if necessary. Depending on the type of surgery and the severity of the disease, a more invasive monitoring must be considered. Although its intraoperative use remains controversial, the pulmonary artery catheter (PAC) provides unique direct continuous measurement of pulmonary arterial pressure, calculation of PVR, and dynamic changes. But, PAC placement may be at higher risk in these patients (arrhythmias and pulmonary artery rupture),11 then the operator’s training is essential. Monitoring with TEE gives direct information on both left and right ventricular filling and performance, provides estimation of PASP,12 and may detect gas embolism during gas insufflation of peritoneal cavity. RV systolic function is assessed with TEE on qualitative measures, such as tricuspidal annular plane systolic excursion, S′ wave (systolic tissue Doppler velocity of the tricuspid annulus), or fractional area change and on visual estimates. TEE has the significant advantage that RV failure can be identified by dynamic rather than the pressure changes, which can be easily missed in PAC measurements. As for PAC, TEE monitoring supposes a skilled operator either for TEE probe insertion or adequate interpretation. Minimally invasive cardiac output monitors (esophageal Doppler and pulse contour analysis devices) have a limited role in these situations because they only detect a low cardiac output without giving specific information about the right heart and pulmonary circulation status.

The induction of general anesthesia and positive-pressure ventilation may lead to hemodynamics decompensation. While etomidate and ketamine seem to be the agents of choice by their little direct effect on vascular resistance, we decided to use target-controlled infusion of propofol because it provides cardiovascular stability and ensures smooth induction, as in heart surgery, and fast recovery.13 Sufentanil was used to blunt cardiovascular response to laryngoscopy and for postoperative pain relief. In addition, mechanical ventilation was set with low tidal volumes and no positive end-expiratory pressure to minimize positive-pressure effect and RV afterload. We had immediate access to iNO in case of PVR elevation. The advantage of iNO is its pulmonary selectivity, and the subsequent reduction in systemic side effects such as systemic hypotension.

Postoperatively, patients with PAH should be monitored in the intensive care unit ≥24 hours because they are at risk of developing worsening PH and RV ischemia as the effects of anesthetics and opioids wear off. Pain increases the PVR, and opiates may have the same effect via their tendency to cause respiratory acidosis. Thereby, arterial and central venous catheters should be removed in the absence of complications and a final TTE must be performed to check PASP and RV function before shifting out to the ward. Above all, PAH therapies must not be discontinued.

Little is known about the perioperative management of patients with PH undergoing laparoscopic surgery.2,3 In the present case, we preferred TEE monitoring to the use of PAC, mainly because our team is better trained in echocardiography. Laparoscopic surgery is classically considered at high risk in these patients: pneumoperitoneum and arterial carbon dioxide elevation can exacerbate PH, and Trendelenburg position acutely reduces pulmonary compliance and increases venous return and RV preload. In this situation, the operative plan was adapted to the clinical status (initially untreated PH) as well the benefits-risks analysis of surgical approach. First, the patient had been worked up and optimized in a specialized center. Second, her functional capacity was improved with medical management, permitting a pulmonary vasodilatory effect that would allow surgery. Third, laparoscopic approach was preferred rather than laparotomy because of its numerous advantages (less intraoperative bleeding, reduced pain, fewer postoperative complications, and shorter hospital stay) and was performed by a well-trained and experienced surgeon. In all cases, insufflation had to be undertaken cautiously, avoiding intraabdominal pressure above 10 mm Hg, high airway pressures during controlled ventilation, and hypercapnia.14 Fourth, it is vital in the perioperative period to have ready, immediate access to iNO or inhaled prostacyclin (iloprost). Finally, appropriate hemodynamic monitoring was crucial, with a special attention to RV function, as well as respiratory monitoring with iterative ABG to optimize mechanical ventilation.

The prognosis of patients with PAH has evolved since the emergence of new therapies. Thorough preoperative assessment and a multidisciplinary discussion in a referral center are essential for medical optimization. Laparoscopic approach in these patients seems not to be contraindicated, providing that there is appropriate anesthesia management and monitoring.

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DISCLOSURES

Name: Olivier Montandrau, MD.

Contribution: This author helped write the manuscript.

Name: Paola Mascitti, MD.

Contribution: This author helped write the manuscript.

Name: Cécile Boucau, MD

Contribution: This author helped write the manuscript.

Name: Julie Cosserat, MD.

Contribution: This author helped review the manuscript.

Name: Christine Denet, MD.

Contribution: This author helped review the manuscript.

Name: Marc Beaussier, MD, PhD.

Contribution: This author helped review the manuscript.

Name: Ivan Philip, MD.

Contribution: This author helped write and review the manuscript.

This manuscript was handled by: Kent H. Rehfeldt, MD.

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