Perioperative management of a patient with haemophilia B and PSVT undergoing radiofrequency ablation: A case report

Abstract

Haemophilia B is a rare inherited bleeding disorder in which patients have impaired coagulation. This study describes a patient with Haemophilia B and paroxysmal supraventricular tachycardia (PSVT) who underwent radio frequency catheter ablation (RFCA). The perioperative replacement therapy with coagulation factor IX (FIX) was agreed upon after an interdisciplinary consultation involving a team of specialists in haematology, cardiovascular medicine and cardiothoracic surgery. There were no obvious bleeding points or complications during the perioperative period following the treatment, nor recurrence of PSVT within a three-year follow-up period. In summary, RFCA can be performed safely in patients with haemophilia B on the premise of developing an individualized perioperative exogenous coagulation factor supplementation regimen based upon an adequate preoperative evaluation and clinical monitoring and management by an interdisciplinary team.

Background

Haemophilia A and B are rare, X-linked bleeding disorders caused by lack or deficiency of clotting Factor VIII (FVIII) or IX (FIX), respectively. ¹ The severity of the disorder depends on the reduction of plasma levels of FVIII or FIX. ¹ Haemophilia A is more common than haemophilia B, with a prevalence of one in 5,000 male live births compared to one in 30,000, respectively. ² The hallmark clinical characteristics manifest early in life as hemarthroses, muscle hematomas or intracranial haemorrhages ¹ . Recurrent bleeding into the joint space results in chronic arthropathy which is associated with substantial long-term morbidity. Intracranial haemorrhages or other significant bleeding can be neurologically devastating or life-threatening. Perioperative management of patients with haemophilia can often be challenging. ³

Paroxysmal supraventricular tachycardia (PSVT) is a common tachyarrhythmia that is sudden in onset and offset. ⁴ Some patients present with palpitations alone, which makes diagnosis difficult. Episodes can last for seconds, minutes, hours or (in rare cases) days. Importantly, when the heart rate increases to 200 beats per minute for long periods of time, cardiac and cerebral blood insufficiency can occur, which will result in low blood pressure, angina pectoris or even heart failure. ⁴ Radiofrequency catheter ablation (RFCA) is recommended as the primary treatment for the long-term management of PVST. ⁵ Its cure rate for atrioventricular nodal reentrant tachycardia (AVNRT) and atrioventricular reentrant tachycardia (AVRT) can exceed 95%, while the incidence of major complications is <1%.^5–7 This present case report describes a male patient with haemophilia  B and PSVT who underwent RFCA. We present this case to emphasise the care required in the perioperative management of similar patients undergoing surgery.

Case Presentation

A 31-year-old male patient was admitted to our hospital with recurrent chest pain. The pain, that had been first experienced by the patient a year earlier, was located in the left precordial region and was accompanied by palpitations, cold sweats, dyspnoea, dizziness and headache. His symptoms lasted for several minutes and then subsided on their own. An hour before admission, the patient had experienced an attack of persistent pain in the precordial region. He was admitted to a cardiology ward for further investigation.

The patient had been diagnosed with congenital haemophilia B at the age of 11 years. Long-term treatment with exogenous coagulation factors had enabled him to maintain his FIX activity level at about 3–5%. The patient’s symptoms included, difficulty in stopping post-traumatic bleeding, slow oozing of blood from wounds and, development of hematomas. Physical examination on admission showed: resting heart rate (RHR), 73 beats per minute; blood pressure, 125/72 mmHg; no significant murmur on cardiac auscultation. His activated partial thromboplastin time (APTT) was 51s, and a complete preoperative coronary computed tomography angiography (CCTA) scan showed no obvious signs of plaque or stenosis in the coronary artery. Cardiac ultrasound showed that his left ventricular ejection fraction (LVEF) was 69%.

On the second day of admission, the patient had another episode of chest pain and was diagnosed with PSVT after review of a bedside electrocardiogram (Figure 1a). His sinus rhythm was restored following treatment with intravenous propafenone (70–mg plus saline 20 ml, 5∼10 min slow bolus, then in 0.5–1.0 mg/min continuous intravenous infusion for 10 minutes). A diagnosis of PSVT was made and elective RFCA was recommended. The patient’s haemophilia necessitated a preoperative, interdisciplinary consultation and so a team of specialists in haematology, cardiovascular medicine and cardiothoracic surgery met to discuss and devise an optimal treatment strategy.

Figure 1.

Examination during hospitalization. (a) Bedside electrocardiogram (ECG) indicated a diagnosis of paroxysmal supraventricular tachycardia (PSVT); (b) Intracardiac electrophysiological examination and (c) The target and actual value of clotting Factor IX (FIX).

A perioperative treatment plan was designed. Firstly, RFCA was to be considered if the coagulation FIX inhibitor assay was negative. The assay was performed based on an increase in FIX following recombinant FIX (rFIX), administration. Secondly, further evaluation of FIX efficacy was required. The patient, who weighed 56 kg, had a basal FIX activity of 5% (moderate range 2–5%). The activity of FIX increased to 52% within half an hour following infusion with 2250 units (IU) of FIX, which resulted in an incremental recovery rate of 118%. Thirdly, contraindications (e.g., infectious diseases (i.e., infective endocarditis; sepsis; pulmonary infections), severe uncontrolled hypertension, electrolyte disturbances, and severe hepatic or renal impairment) were excluded. To reduce the risk of bleeding, the required FIX dose was calculated based on an increase of the recovery rate of 0.8 IU/dl (according to guidelines, ³ 1.00 IU/kg of rFIX was expected to increase FIX levels in plasma by 0.4–1.4 IU/dl, with an average increase of 0.8%, and so FIX 7250 IU was infused one hour prior to surgery).

Electrodes were surgically placed via the right and left femoral vein, in the coronary sinus, the bowel of Hirschsprung, and the right ventricular site. The procedure, as suggested by intracardiac electrophysiological examination, was over a left-sided bypass tract (Figure 1b). Postoperatively, the patient had FIX levels measured each morning and was treated daily with rFIX during hospital admission to maintain FIX at target values (Figure 1c). The FIX level was maintained for one week after surgery (Table 1). The dose of FIX infusion was adjusted according to the monitoring results and target concentration. On postoperative Day 7, no obvious haemorrhagic foci were observed in a cardiac ultrasound, no obvious thrombus was found in a lower extremity vascular ultrasound, and the incision had healed well.

Table 1.

Perioperative clotting Factor IX (FIX) target values and drug dosage.

Time	Target value*	Frequency of medication	Dose of FIX (IU)γ
1 hour before surgery	100%	once	7250
12 hours post-surgery	100%	once	5750
24 hours post-surgery	100%	once	5750
2–5 days post-surgery	70%	once every 12 hours	4000
5–7 days post-surgery	50%	once per day	2000

*

Normal FIX levels are typically 50–150%.

γ Dosage of FIX (IU) = body weight (kg) × target value of FIX (%) × inverse of the observed recovery rate.

For this patient, reaching his left heart through the femoral vein pathway required an incision of the interatrial septum and could have resulted in pericardial perforation or even tamponade. While the risk of bleeding was greater than risk of thrombosis, we did not administer routine anticoagulation therapy post-surgery. Instead, we prevented thrombosis by a stepwise reduction of the coagulation factor dosage and suspension of drug administration for seven days post-surgery. The patient had no recurrence of chest pain during a three-year follow-up period, which greatly improved his quality of his life (Figure 2).

Figure 2.

Patient’s electrocardiogram (ECG) taken at 11-month follow-up visit showing normal sinus rhythm.

The reporting of this study conforms to CARE guidelines.8 Written informed consent was obtained from the patient to publish his anonymised data. The study was approved by the Research Ethics Committee of Shulan (Hangzhou) Hospital (approval number KY2023028).

Discussion

As illustrated by this case study, people with haemophilia B undergoing surgery require an individual perioperative exogenous coagulation factor supplementation regimen based upon adequate preoperative evaluation. Haemostatic support with replacement FIX preoperatively will ensure intraoperative haemostasis and levels of FIX should be monitored in the postoperative period to minimise bleeding and ensure wound healing.

This report describes a middle-aged man with recurrent chest pain and palpitations, symptoms typical of PVST, which was definitively confirmed by diagnosis. Controlling his symptoms with medication could only be used as a short-term treatment, which resulted in his being a candidate for PVST.^5–7 However, he had a high risk of bleeding during perioperative period because of his haemophilia. Although gene therapy for haemophilia B has progressed and evolved over recent years ⁹ , coagulation factor replacement therapy remains the primary perioperative treatment option for these patients. ¹⁰ Indeed, life-long FIX replacement therapy with intravenous plasma-derived FIX (pdFIX) or rFIX concentrates is the standard of care for patients with haemophilia B. ¹¹

Despite a literature search, we found no previous reports of RFCA in patients with haemophilia and no consensus guidelines on the effective perioperative management of haemophiliacs undergoing cardiac surgery. Although limited, we did find several case reports describing cardiac surgery in patients with haemophilia. In one report, based on data from six cases of cardiac surgery performed in patients with haemophilia A, intermittent high-dose clotting factor infusion was used in their perioperative management, and none developed postoperative haemorrhagic complications. ¹² In other reports, describing cardiac procedures (e.g., on‐pump coronary artery bypass grafting (CABG), aortic valve replacement (AVR) and mitral valve repair) in patients with haemophilia B, while standardized programs for intraoperative management were not provided, a target level of 100% FIX post-operatively was generally accepted.^13,14 Although some of the patients developed bleeding complications, their survival outcomes were good.

In our case, the lack of data led us to initiate an interdisciplinary team to determine a surgical and postoperative maintenance treatment plan for our patient. Initially, the patient underwent FIX inhibitor examination to determine the feasibility of coagulation factor replacement. Further evaluation of FIX efficacy followed, that ensured the safety of a surgical procedure involving arteriovenous puncture in this patient. A high target value (FIX 100%) was formulated during the intraoperative and two-day postoperative period to reduce deep tissue bleeding. Post-operatively, treatment with FIX was maintained using a stepwise reduction in the supplementation of the exogenous coagulation factor. In the immediate postoperative period, the FIX level of our patient was 118%, and he had no bleeding complications.

In summary, the success of this case indicates that RFCA can be performed safely and efficiently in patients with haemophilia B on the premise of developing an individualized perioperative exogenous coagulation factor supplementation regimen based upon an adequate preoperative evaluation and clinical monitoring and management by an interdisciplinary team.