Anaesthetic management of a parturient with the postural orthostatic tachycardia syndrome: a case report

Abstract

Postural orthostatic tachycardia syndrome encompasses a group of disorders characterized by orthostatic intolerance. We describe the anaesthetic management of analgesia for labour and of Caesarean section in a parturient suffering from this disorder. Worsening of her symptoms during pregnancy was managed with an increase in the dose of beta-blockers taken by the patient. Epidural analgesia was instigated early to attenuate the stress of labour and avoid consequent triggering of a tachycardic response. Slow titration of epidural analgesia and anaesthesia after an adequate fluid preload was undertaken to minimize hypotension and subsequent tachycardia. Neuraxial opioid, combined with non-steroidal anti-inflammatory drugs and bilateral iliohypogastric and ilioinguinal nerve blocks were used to optimize postoperative analgesia.

Perioperative Management of Ehlers-Danlos Type III Syndrome Associated With Postural Orthostatic Tachycardia in Patients Undergoing General Anesthesia

“…A second intraoperative concern is invasive hemodynamic monitoring for continuous blood pressure measurement. Although invasive monitoring places patients with EDS III at risk for vascular damage, it may be valuable in the management of POTS given the potential for rapid hemodynamic changes, as seen in both of our patients [ 1 , 16 ] Further, if hemodynamic instability occurs without invasive monitoring, it would be difficult and potentially traumatic to establish arterial access quickly, even with ultrasound guidance [ 4 ]. For our cases, one was monitored with a non-invasive cuff and the other had a post-induction ultrasound-guided arterial line.…”

“…Positioning recommendations continue postoperatively as well as avoidance of skin trauma from subcutaneous and intramuscular injections, with quick removal of intravenous lines or invasive monitoring devices [ 16 ]. Patients with EDS III should be monitored for high risk of surgical emphysema secondary to accidental tracheal puncture and pleural effusion or tamponade from central venous catheters [ 16 ]. Pain management can also be challenging when relying on regional analgesia, as it has been found that patients with EDS may be resistant to local anesthetics [ 11 ].…”

Perioperative Management of Ehlers-Danlos Type III Syndrome Associated With Postural Orthostatic Tachycardia in Patients Undergoing General Anesthesia

“…A second intraoperative concern is invasive hemodynamic monitoring for continuous blood pressure measurement. Although invasive monitoring places patients with EDS III at risk for vascular damage, it may be valuable in the management of POTS given the potential for rapid hemodynamic changes, as seen in both of our patients [ 1 , 16 ] Further, if hemodynamic instability occurs without invasive monitoring, it would be difficult and potentially traumatic to establish arterial access quickly, even with ultrasound guidance [ 4 ]. For our cases, one was monitored with a non-invasive cuff and the other had a post-induction ultrasound-guided arterial line.…”

“…Positioning recommendations continue postoperatively as well as avoidance of skin trauma from subcutaneous and intramuscular injections, with quick removal of intravenous lines or invasive monitoring devices [ 16 ]. Patients with EDS III should be monitored for high risk of surgical emphysema secondary to accidental tracheal puncture and pleural effusion or tamponade from central venous catheters [ 16 ]. Pain management can also be challenging when relying on regional analgesia, as it has been found that patients with EDS may be resistant to local anesthetics [ 11 ].…”

A Case Report and Review of Postural Orthostatic Tachycardia Syndrome in Pregnancy

“…Many women with POTS have been able to undergo safe vaginal delivery, 3 4 5 7 11 even without anesthesia. 4 The presence of POTS should generally not influence the choice of whether to deliver vaginally or by cesarean section.…”

“…In some cases it may be important to establish analgesia early in labor to prevent a tachycardic response. 7 9 It has been shown that nonpregnant patients with POTS experience a relatively larger decrease in blood pressure in the early phases of Valsalva, accompanied by a subsequently large increase in blood pressure and heart rate in the later phases. McEvoy et al, who examined the anesthetic management of POTS in delivery, suggested that in the pregnant patient these hemodynamic changes could increase pelvic blood vessel resistance thereby decreasing uteroplacental blood flow.…”

“…Phenylephrine is commonly used to treat hypotension associated with neuraxial blockade in POTS patients. 7 9 Although a vasoconstricting agent (similar to midodrine), teratogenicity, and lactation are not a major concern, as this agent is generally used intrapartum and in a short exposure window.…”

Perioperative Management of Ehlers-Danlos Type III Syndrome Associated With Postural Orthostatic Tachycardia in Patients Undergoing General Anesthesia

“…A second intraoperative concern is invasive hemodynamic monitoring for continuous blood pressure measurement. Although invasive monitoring places patients with EDS III at risk for vascular damage, it may be valuable in the management of POTS given the potential for rapid hemodynamic changes, as seen in both of our patients [ 1 , 16 ] Further, if hemodynamic instability occurs without invasive monitoring, it would be difficult and potentially traumatic to establish arterial access quickly, even with ultrasound guidance [ 4 ]. For our cases, one was monitored with a non-invasive cuff and the other had a post-induction ultrasound-guided arterial line.…”

“…Positioning recommendations continue postoperatively as well as avoidance of skin trauma from subcutaneous and intramuscular injections, with quick removal of intravenous lines or invasive monitoring devices [ 16 ]. Patients with EDS III should be monitored for high risk of surgical emphysema secondary to accidental tracheal puncture and pleural effusion or tamponade from central venous catheters [ 16 ]. Pain management can also be challenging when relying on regional analgesia, as it has been found that patients with EDS may be resistant to local anesthetics [ 11 ].…”

A Case Report and Review of Postural Orthostatic Tachycardia Syndrome in Pregnancy

“…Many women with POTS have been able to undergo safe vaginal delivery, 3 4 5 7 11 even without anesthesia. 4 The presence of POTS should generally not influence the choice of whether to deliver vaginally or by cesarean section.…”

“…In some cases it may be important to establish analgesia early in labor to prevent a tachycardic response. 7 9 It has been shown that nonpregnant patients with POTS experience a relatively larger decrease in blood pressure in the early phases of Valsalva, accompanied by a subsequently large increase in blood pressure and heart rate in the later phases. McEvoy et al, who examined the anesthetic management of POTS in delivery, suggested that in the pregnant patient these hemodynamic changes could increase pelvic blood vessel resistance thereby decreasing uteroplacental blood flow.…”

“…Phenylephrine is commonly used to treat hypotension associated with neuraxial blockade in POTS patients. 7 9 Although a vasoconstricting agent (similar to midodrine), teratogenicity, and lactation are not a major concern, as this agent is generally used intrapartum and in a short exposure window.…”

Perioperative Management of Ehlers-Danlos Type III Syndrome Associated With Postural Orthostatic Tachycardia in Patients Undergoing General Anesthesia

“…A second intraoperative concern is invasive hemodynamic monitoring for continuous blood pressure measurement. Although invasive monitoring places patients with EDS III at risk for vascular damage, it may be valuable in the management of POTS given the potential for rapid hemodynamic changes, as seen in both of our patients [ 1 , 16 ] Further, if hemodynamic instability occurs without invasive monitoring, it would be difficult and potentially traumatic to establish arterial access quickly, even with ultrasound guidance [ 4 ]. For our cases, one was monitored with a non-invasive cuff and the other had a post-induction ultrasound-guided arterial line.…”

“…Positioning recommendations continue postoperatively as well as avoidance of skin trauma from subcutaneous and intramuscular injections, with quick removal of intravenous lines or invasive monitoring devices [ 16 ]. Patients with EDS III should be monitored for high risk of surgical emphysema secondary to accidental tracheal puncture and pleural effusion or tamponade from central venous catheters [ 16 ]. Pain management can also be challenging when relying on regional analgesia, as it has been found that patients with EDS may be resistant to local anesthetics [ 11 ].…”

A Case Report and Review of Postural Orthostatic Tachycardia Syndrome in Pregnancy

“…Many women with POTS have been able to undergo safe vaginal delivery, 3 4 5 7 11 even without anesthesia. 4 The presence of POTS should generally not influence the choice of whether to deliver vaginally or by cesarean section.…”

“…In some cases it may be important to establish analgesia early in labor to prevent a tachycardic response. 7 9 It has been shown that nonpregnant patients with POTS experience a relatively larger decrease in blood pressure in the early phases of Valsalva, accompanied by a subsequently large increase in blood pressure and heart rate in the later phases. McEvoy et al, who examined the anesthetic management of POTS in delivery, suggested that in the pregnant patient these hemodynamic changes could increase pelvic blood vessel resistance thereby decreasing uteroplacental blood flow.…”

“…Phenylephrine is commonly used to treat hypotension associated with neuraxial blockade in POTS patients. 7 9 Although a vasoconstricting agent (similar to midodrine), teratogenicity, and lactation are not a major concern, as this agent is generally used intrapartum and in a short exposure window.…”