Anesthetic management of an adult female with uncorrected univentricular heart and comorbid ectopic pregnancy for unilateral salpingectomy: a case report

Abstract

Background

Anesthesia management for patients with uncorrected univentricular heart and ectopic pregnancy undergoing emergency laparotomy presents significant challenges. Close collaboration among a multidisciplinary team, a keen understanding of anatomy and pathophysiology, and optimization of intraoperative hemodynamic targets are crucial for successful outcomes.

Case presentation

We report the anesthetic challenges encountered in a case of an adult female with uncorrected univentricular heart and ectopic pregnancy who required urgent exploratory laparotomy. We summarize critical anesthetic considerations for patients with univentricular heart physiology undergoing non-cardiac surgery. The patient successfully underwent unilateral salpingectomy under general anesthesia.

Conclusions

Multidisciplinary coordination and tailored hemodynamic management were pivotal to successful outcomes, providing valuable reference for the clinical diagnosis and treatment of similar cases in the future.

Background

A univentricular heart is a rare congenital heart defect, accounting for approximately 3% of all congenital heart diseases (CHD) [1]. It includes several conditions [2]: double inlet atrioventricular (AV) connections (either double inlet left ventricle [DILV] or double inlet right ventricle), the absence of one AV connection (such as mitral or tricuspid atresia [TA]), a common AV valve with only one well-developed ventricle (as seen in unbalanced common AV canal defects), a well-developed single ventricle associated with heterotaxy syndrome (single ventricle heterotaxy syndrome) and hypoplastic left heart syndrome (HLHS). Univentricular physiology creates a parallel circulation in which a single ventricle pumps mixed venous blood to both the systemic and pulmonary circuits. This results in chronic cyanosis and requires a precise balance between pulmonary (Qp) and systemic (Qs) blood flow. Any factor that alters vascular resistance (e.g., hypotension, hypoxia, or hypercapnia) can quickly destabilize the Qp: Qs ratio, which can lead to pulmonary overflow or systemic hypoperfusion [2, 3].

The mortality for uncorrected univentricular hearts is very high. A retrospective study in Norway [4, 5] revealed that, between 1990 and 2015, the mortality within the first month of life for patients with uncorrected univentricular heart was as high as 83.9% (125/149), with only 2 patients surviving beyond this period (2/151). Survival into adulthood without surgical correction has only been reported in a few case studies [6, 7]. Reports of non-cardiac surgery in adults with uncorrected univentricular hearts are rare. Thus, more clinical experience is needed to provide reference for future clinical diagnosis and treatment processes. Accordingly, we present a case of a complex uncorrected univentricular heart associated with an ectopic pregnancy, which underwent a salpingectomy. We also review previously published cases of non-cardiac surgeries performed in patients with uncorrected univentricular hearts. Additionally, we discuss important considerations for anesthetic management in these patients, emphasizing the significance of understanding the pathophysiology of univentricular hearts and the role of a multidisciplinary team in ensuring optimal hemodynamic stability during the perioperative period.

Case presentation

Patient information

A 28-year-old female (40 kg) with uncorrected complex CHD presented with 52 days of amenorrhea accompanied by progressive abdominal pain and vaginal bleeding. She had a history of receiving general anesthesia for an abortion, which she tolerated without major complications. However, detailed records from that procedure were unavailable. She was not under long-term cardiology follow-up, nor was she taking any cardiac medications prior to admission. Her uncorrected CHD (echocardiography findings below) resulted in lifelong cyanosis, a baseline oxygen saturation (SpO₂) of 78%, and a New York Heart Association (NYHA) class III functional status, consistent with a functional capacity of fewer than four MET tasks. Surgical correction had been deferred in childhood due to parental concerns regarding the perceived high risk of surgery and financial constraints.

On admission, elevated beta-human chorionic gonadotropin (β-HCG) (3194 mIU/L) and transvaginal ultrasound revealed a ruptured left adnexal mass, consistent with ectopic pregnancy. Multidisciplinary consensus recommended emergency laparotomy. Ethics approval from the Institutional Review Board of the Affiliated Hospital of Jining Medical University (2024-03-C018) was obtained for the publication of this case report.

Clinical and laboratory findings

The patient exhibited marked cyanosis (lips, nail beds), digital clubbing, and tachycardia (HR 95 bpm). Transthoracic echocardiography demonstrated situs inversus, dextrocardia, univentricular heart, complete atrioventricular canal defect (Fig. 1 A), transposition of the great arteries, double aortic arch, and bilateral superior vena cava (Fig. 1 C and Fig. 1 D). The univentricular heart was anatomically a left ventricle with underdevelopment of the right ventricle. Moderate regurgitation of the common atrioventricular valve (Fig. 1 B) was observed, with a peak regurgitant velocity of 4.8 m/s. Pulmonary valve stenosis was also noted, with a peak velocity of 3.8 m/s. Coagulopathy (prothrombin time (PT) 16.4 s, international normalized ratio (INR) 1.33) and polycythemia (hemoglobin (Hb) 221 g/L, hematocrit (Hct) 66.1%) were noted. Arterial blood gas revealed hypoxemia (partial pressure of oxygen (PaO₂) 46 mmHg) and compensated metabolic acidosis (pH 7.30, lactate 0.6 mmol/L).

Fig. 1.

Imaging of the patient’s heart. A Images of the patient’s complete atrioventricular canal defect. B Images of the patient’s common atrioventricular valve regurgitation. C Images of the patient’s common ventricular origin of the aorta and pulmonary artery. D Images of the patient’s double aortic arch LA = left atrium; RA = right atrium; SV = single ventricle

We summarized the specific issues presented by the patient to facilitate the development of an individualized anesthetic plan during the procedure. (Table 1)

Table 1.

Considerations related to patient, anesthesia, and surgery

Patient-related issues	Univentricular physiology
	Chronic cyanosis and polycythemia
	Coagulation abnormalities
	Potential excessive blood loss due to ruptured ectopic pregnancy
Anesthesia-related issues	Vascular resistance systemic decreased during induction
	Myocardial contractility suppression by anesthetic agents
	Hypoventilation/hypercapnia may affect hemodynamics, increasing pulmonary artery pressure
	Mechanical ventilation may reduce venous return
Surgery-related issues	Laparoscopic surgery can reduce venous return
	Intra-abdominal hematoma compressing the inferior vena cava, with further reduction in venous return after abdominal incision
	Risk of endocarditis

Operative and anesthetic management

Comprehensive invasive hemodynamic monitoring was established before general anesthesia was induced. Prior to induction, a left radial arterial line and a central venous line were placed. A manual defibrillator with paddles was confirmed to be immediately available in the operating room as the primary backup for arrhythmia management. General anesthesia was selected due to hemodynamic instability and coagulopathy. Induction with sufentanil (20 µg), midazolam (3 mg), rocuronium (40 mg), and etomidate (4 mg) achieved rapid loss of consciousness. Mechanical ventilation was initiated with an FiO₂ of 1.0. Our hemodynamic management strategy aimed to maintain oxygen saturation at the patient’s preoperative baseline level (78%−85%), while avoiding a significant increase that could indicate pulmonary over circulation at the expense of systemic output. Her SpO₂ remained within this target range (79%−85%) throughout the procedure.

Upon entering the abdominal cavity, approximately 800 mL of clotted blood was evacuated, confirming significant intra-abdominal hemorrhage. Immediately following peritoneal incision and decompression, the patient developed profound hypotension (82/40 mmHg) with tachycardia (HR 160 bpm). This was consistent with an acute reduction in preload due to the sudden loss of intra-abdominal pressure.

The initial management targeted hemodynamic stabilization. A phenylephrine bolus (40 µg) was administered to rapidly restore systemic vascular resistance and coronary perfusion pressure. This stabilized the blood pressure at 90/60 mmHg and reduced the heart rate to 120 bpm. Concurrently, fluid resuscitation was initiated with a 200 mL bolus of compound sodium chloride solution, which increased the CVP from 12 cmH₂O to 14 cmH₂O.

However, the patient experienced a recurrence of hypotension, indicating a persistent volume deficit and limited preload reserve. An additional 600 mL of fluid was administered, raising the CVP further to 16 cmH₂O. The observed dissociation between the rising CVP and the persistent hemodynamic instability highlighted the ventricle’s preload intolerance and diastolic dysfunction. The patient’s response underscored the importance of afterload support with phenylephrine in this setting, with fluid administration playing a secondary, supportive role.

Nevertheless, the persistence of hypotension despite a rising CVP indicated a complex hemodynamic state in which traditional indicators of volume status were unreliable. This likely reflected the limited preload reserve and diastolic dysfunction of the single ventricle. In this condition, further fluid administration increased intracardiac pressures without improving effective circulating volume or cardiac output.

Although the patient was asymptomatic preoperatively, a double aortic arch carries lifelong risks of tracheoesophageal compression. Potential manifestations include stridor, dysphagia, or recurrent pneumonia [8]. Perioperatively, airway edema or hematoma could acutely exacerbate compression.

Postoperative signs

The patient was extubated and transferred to the intensive care unit (ICU) for continuous invasive hemodynamic monitoring and support. A multimodal analgesia regimen combining ropivacaine wound infiltration with sufentanil intravenous infusion provided effective pain control, enabling discharge on postoperative day four.

Follow-up

During the seven-day postoperative follow-up, the patient reported no adverse events. Six months post-discharge, the patient presented with hemoptysis. Given the patient’s history of chronic cyanosis, the hemoptysis was presumed to be secondary to pulmonary arteriovenous malformations (PAVMs). The patient was treated conservatively with supplemental oxygen, systemic tranexamic acid, and avoidance of anticoagulants, resulting in partial resolution of symptoms. A definitive diagnostic procedure (e.g., CT angiography or cardiac catheterization) was not performed during the acute episode. Twelve months postoperatively, progressive cyanosis (resting SpO₂ of 76%) prompted surgical intervention. She underwent a bidirectional cavopulmonary anastomosis (Glenn procedure). Intraoperative direct measurement revealed a critically low pulmonary artery pressure of 8 mmHg, indicating favorably low pulmonary vascular resistance. Postoperatively, she experienced sustained clinical improvement, with oxygen saturation typically ranging from the mid-80s to approximately 90% on room air. The patient was discharged with stable hemodynamics and no perioperative complications.

Review of previous studies

We searched for reports on uncorrected univentricular heart patients undergoing non-cardiac surgery (Table 2). We identified 6 case reports involving a total of 8 patients with uncorrected univentricular hearts who underwent various non-cardiac surgeries [9–14]. Among them, 5 patients underwent cesarean Sects [10, 12, 13]., including both emergency and twin pregnancy cesarean deliveries. One patient had an abortion procedure [11], one underwent an appendectomy [9], another had emergency craniotomy for brain abscess removal [14]. All surviving patients with uncorrected univentricular heart were female, aged between 20 and 40 years, with NYHA classifications ranging from II to III. Except for one patient [13], the remaining 7 patients had varying degrees of pulmonary hypertension. All 8 patients exhibited some degree of cyanosis, with preoperative oxygen saturation levels ranging from 68% to 92%. The anesthetic approaches for non-cardiac surgeries in these patients varied: Among the 5 cesarean sections, 4 were performed under combined spinal-epidural anesthesia [10, 12, 13], while 1 was performed under general anesthesia [12]. The patient with uncorrected univentricular heart who had an appendectomy received intravenous anesthesia combined with regional nerve block [9]. The patient who underwent craniotomy for intracranial abscess removal was managed under general anesthesia [14]. Multidisciplinary teams (MDT) were involved in 90% of obstetric cases, including cardiologists, obstetricians, anesthesiologists, and neonatologists. MDT planning was critical for optimizing hemodynamics and neonatal outcomes. The majority of cases (75%) involved a univentricular left ventricle morphology, often accompanied by transposition of the great arteries (58%) and pulmonary stenosis (PS, 67%). Pulmonary stenosis emerged as a critical compensatory mechanism, limiting pulmonary blood flow and reducing ventricular volume overload, thereby prolonging survival. None of the patients underwent transesophageal echocardiography (TEE). No perioperative deaths were reported in these patients, and all survived.

Table 2.

Published case reports: non-cardiac surgery in patients with uncorrected univentricular heart

Author	Anomaly	Age	NYHA	Pulmonary stenosis	Cyanosis	SPO2	Anesthesia	Whether multidisciplinary case discussion conducted	Was esophageal ultrasound performed	Outcome	Surgery
Hai-Zhen Duan [11]	Single atrium and univentricular heart with pulmonary valve stenosis; left ventricle morphology; right-sided univentricular heart	40	NA	Present	Present	68	None	NA	NA	Survived	Abortion
Yu Wu [9]	Single atrium and univentricular heart with pulmonary valve stenosis, common atrioventricular valve, moderate atrioventricular valve regurgitation	20	III	Present	Present	72%	Intravenous anesthesia and regional nerve block anesthesia	Yes	NA	Survived	Appendicectomy
Gurpreet Kaur [14]	Univentricular heart with left ventricular morphology, mitral valve stenosis; moderate tricuspid regurgitation, transposition of the great arteries, pulmonary valve stenosis	22	NA	Present	Present	85%	General anesthesia	NA	NA	Survived	Emergency craniotomy for brain abscess removal
Yu Du [10]	Univentricular heart (with dominant left ventricle), atrioventricular valve malposition, double-inlet left ventricle, abnormal positioning of the great arteries, pulmonary artery stenosis, atrial septal defect, and left-to-right shunt.	35	II	Present	Present	85%	Combined spinal-epidural anesthesia	Yes	NA	Survived	Twin pregnancy cesarean section
Jill Fong [13]	Univentricular heart, L-type transposition of the great arteries (L-TGA), ventricular inversion, atrial septal defect, right atrioventricular valve atresia, mild subvalvular and valvular stenosis	29	II	Present	Present	86	Combined spinal-epidural anesthesia	NA	NA	Survived	Emergency cesarean section
Kana Wang [12]	Univentricular heart (left-type) with double outlet; severe pulmonary valve regurgitation; rightward malposition of the aortic arch; moderate tricuspid and mitral valve regurgitation	26	I	NA (severe pulmonary regurgitation)	Present	80	Combined spinal-epidural anesthesia	Yes	NA	Survived	Cesarean section
Kana Wang [12]	Single left ventricle, tricuspid atresia, pulmonary stenosis, atrial septal defect with bidirectional shunt, mural thrombus in the left atrium.	20	III	Present	Present	86	Combined spinal-epidural anesthesia	Yes	NA	Survived	Cesarean section
Kana Wang [12]	Single right ventricle with double outlet (ejection fraction [EF] = 52%), mitral valve atresia, atrial septal defect with left-to-right shunt, pulmonary valve stenosis	34	II-III	Present	Present	82	General anesthesia	Present	NA	Survived	Cesarean section

Discussion

The anesthetic management of patients with uncorrected univentricular heart physiology presents formidable challenges, necessitating meticulous multidisciplinary coordination to address their unique hemodynamic vulnerabilities. These individuals exhibit complex circulatory adaptations, including limited cardiac reserve and altered preload-afterload relationships, which predispose them to profound intraoperative instability. Our case highlights these challenges in a 28-year-old woman with situs inversus, dextrocardia, and univentricular left ventricle physiology undergoing emergency laparotomy for ruptured ectopic pregnancy.

Patients with single-ventricle physiology rarely survive into adulthood without palliative surgery. However, certain anatomical configurations—such as a dominant left ventricle with moderate pulmonary stenosis (PS)—may permit compensated circulation by balancing systemic and pulmonary blood flow. In our patient, PS (peak gradient: 59 mmHg) served as a protective mechanism, preventing pulmonary over circulation while maintaining adequate systemic perfusion. This aligns with prior observations that adults with balanced circulation and preserved ventricular function (EF 59% in our case) may experience delayed symptom onset despite uncorrected anatomy [7, 15, 16]. The absence of Fontan palliation further underscores the precarious equilibrium in such patients, where even minor hemodynamic perturbations can precipitate decompensation.

The choice of open laparotomy over laparoscopic techniques was driven by the imperative for rapid hemorrhage control and avoidance of pneumoperitoneum-induced hemodynamic compromise. Increased intra-abdominal pressure during laparoscopy could exacerbate preexisting venous return limitations in single-ventricle physiology, potentially triggering catastrophic hypotension [3]. General anesthesia was prioritized over neuraxial techniques due to coagulopathy (INR 1.33) and the need for precise hemodynamic control.

Anesthetic induction with etomidate and sufentanil minimized myocardial depression while suppressing sympathetic responses to laryngoscopy. Maintenance with sevoflurane permitted rapid titration to surgical stimuli, though its negative inotropic effects necessitated cautious use. Hemodynamic stability was further challenged post-peritoneal incision, with hypotension (82/40 mmHg) and tachycardia (HR 160 bpm) attributable to multifactorial mechanisms:

Preload Reduction: Abdominal decompression abruptly decreased intra-abdominal pressure, causing venous pooling and reduced cardiac filling—a phenomenon analogous to post-decompression physiology in abdominal compartment syndrome [17].

Sympathetic Activation: Surgical nociception triggered adrenergic surges, exacerbating myocardial oxygen demand in a heart already compromised by chronic hypoxemia.

Fluid-Responsive Limitations: Despite aggressive resuscitation with 800 mL compound sodium chloride (CVP increased from 12 to 16 cmH₂O), single-ventricle anatomy restricted preload tolerance, as elevated CVP reflected ventricular diastolic dysfunction rather than effective volume expansion.

Interpreting the hemodynamic changes in this case was challenging. The occurrence of refractory hypotension alongside an elevated CVP following fluid administration highlights the limitations of conventional monitoring in single-ventricle physiology. This phenomenon could be attributable to several mechanisms, including diastolic dysfunction, volume overload, worsening atrioventricular valve regurgitation, or myocardial stunning. Without TEE, it was impossible to definitively discriminate between these possibilities. TEE would have provided an invaluable, real-time assessment of ventricular filling, contractility, and valvular function. This would have allowed for more precise guidance of fluid therapy, inotropic support, and vasopressor selection. This case strongly underscores the recommendation to use TEE in all high-risk non-cardiac surgeries involving patients with complex CHD to optimize hemodynamic management.

Although the use of 100% inspired oxygen during induction is theoretically risky in single-ventricle physiology due to its potential to reduce pulmonary vascular resistance and increase pulmonary blood flow, it was deemed necessary in this emergency context. The need to maximize oxygen reserve during rapid sequence induction in a hypovolemic, cyanotic patient took precedence. The patient’s fixed pulmonary stenosis had a protective effect, as evidenced by the stability of her intraoperative oxygen saturation within her baseline range.

Notably, the absence of TEE hindered real-time assessment of ventricular filling and contractility. In similar high-risk cases, TEE has proven invaluable for guiding fluid therapy and inotropic support, particularly in patients with complex cardiac anatomy [3]. Therefore, we strongly recommend using TEE to optimize hemodynamic management in such high-risk scenarios.

This case underscores the critical role of interdisciplinary collaboration. Cardiologists provided essential insights into ventricular tolerance thresholds, while obstetricians prioritized expeditious surgical intervention to mitigate hemorrhagic shock. The patient’s subsequent cavopulmonary anastomosis at 12 months postoperatively exemplifies the longitudinal care required for single-ventricle patients, as progressive cyanosis ultimately necessitated definitive palliation.

Conclusion

In the perioperative management of adults with an uncorrected univentricular heart undergoing non-cardiac surgery, the underlying pathophysiology is crucial. This includes the fragile equilibrium of the pulmonary-to-systemic flow ratio (Qp: Qs) and protective compensatory mechanisms such as pulmonary stenosis. For these high-risk patients, TEE is strongly recommended to assess hemodynamics in real time. Standardized, multidisciplinary protocols that integrate cardiology, anesthesiology, and surgical expertise must be implemented throughout the perioperative duration.

Authors’ contributions

All authors contributed to the study conception and design. Writing - original draft preparation: [Jiaying Xin]; Writing - review and editing: [Xuebo Bai]; Conceptualization: [Xuebo Bai, Jiaying Xin, Jianguo Wang]; Methodology: [Xuebo Bai, Jiaying Xin, Jianguo Wang, Laizhu Zhang]; Formal analysis and investigation: [Xuebo Bai, Jiaying Xin, Jianguo Wang, Laizhu Zhang]; Resources: [Xuebo Bai, Jiaying Xin]; Supervision: [Laizhu Zhang], and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Funding

The study did not receive any specific funding from funding agencies in the public, commercial, or non-profit sectors.

Data availability

The data used to support the findings of this study are included within the article.

Declarations

Ethics approval and consent to participate

Ethics approval was obtained from the Affiliated Hospital of Jining Medical University (2024-03-C018).

Consent for publication

Written informed consent for the publication of their clinical details was obtained from the patient. A copy of the consent form is available for review by the Editor of this journal.

Competing interests

The authors declare no competing interests.