Surgical technique challenges in adult aortic coarctation patients: A case series

Abstract

Introduction and importance

Aortic coarctation (CoA) is relatively rare in adult patients and often presents with non-specific symptoms. It typically results from either delayed recognition or residual lesion following incomplete repair in childhood. While concomitant cardiac abnormalities are commonly detected in children, adult patients more often present with isolated CoA or complications resulting from late diagnosis. Untreated CoA can lead to significant long-term complications, including refractory hypertension, heart failure, and aortic aneurysm or rupture.

Case series

We present a case series of three adult patients with CoA of varying severity and associated cardiac pathologies. Each patient underwent a different open surgical approach. First, a 39-year-old male presented with recurrent CoA with severe adhesions. Second, a 48-year-old male presented with proximal descending CoA accompanied by ascending aortic aneurysm and severe aortic stenosis. Third, a 25-year-old male presented with CoA and severe aortic valve insufficiency. All patients were diagnosed with CoA via echocardiography and underwent surgical procedures.

Discussion

Managing adult CoA, particularly when combined with other cardiac pathologies, poses significant surgical challenges due to the lack of standardized guidelines for such complex conditions. Surgeons must make critical decisions regarding the order of repairs, the selection of the most suitable surgical approach, and the ideal timing of intervention to ensure patient safety.

Conclusion

Surgical approaches for CoA are individualized based on the complexity of the coarctation and associated cardiac pathologies. Surgical management of CoA either in adults or neonates should be performed accurately and effectively to reduce morbidity and long-term complications.

Highlights

• •Adult aortic coarctation (CoA) presents unique surgical challenges due to late diagnosis and varied pathology.
• •Individualized surgical approach led to successful outcomes in all three adult CoA cases.
• •Techniques used included extra-anatomical bypass, Bental procedure, and patch aortoplasty.
• •Single-stage open repair was feasible and effective despite complex comorbidities.
• •Lifelong monitoring is essential to detect late complications such as restenosis or aneurysm.

1. Introduction

Coarctation of the aorta (CoA) is a congenital heart defect (CHD) present in 5–8 % of CHD cases, marked by a localized or segmental narrowing of the aortic lumen. The obstruction typically arises near the ductus arteriosus attachment site, often just beyond the left subclavian artery [1]. The exact cause of CoA is not entirely understood, but genetic factors are believed to play a role. CoA is often associated with other congenital heart defects, such as bicuspid aortic valve, ventricular septal defect (VSD), atrial septal defect (ASD), persistent ductus arteriosus (PDA), transposition of the great arteries (TGA), and other cardiac conditions [2,3].

Currently, neonatal screening and early management have led to the majority of CoA cases being diagnosed and treated in the neonatal period. However, in some individuals, CoA may not be diagnosed until adulthood. This diagnostic delay leads to underrecognition of asymptomatic cases, resulting in adult-onset detection and potential underestimation of congenital prevalence [5]. In adults, CoA classically identified through paired diminished/absent femoral pulses and hypertension in the upper extremities [4]. Prognosis is severely impacted by late diagnosis, which untreated patients have a median survival of only 35 years, with 90 % mortality by age 50 due to complications like aortic rupture or heart failure [6].

CoA is usually diagnosed through physical examination, electrocardiography (ECG), echocardiography, computed tomography (CT), or magnetic resonance imaging (MRI) [7]. Managing CoA in adult patients concomitant with other cardiac pathologies give a significant surgical challenge due to the absence of standardized guidelines for such complex conditions. Surgeons should make critical decisions regarding the order of repairs, the selection of the most suitable surgical approach, and the ideal timing for intervention [8]. This study aims to describe the surgical techniques used in three adult CoA patients treated with open surgery (Table 1).

Table 1.

Patient characteristics and surgical indications.

Patient	Age (years)	Gender	Clinical presentation	Surgical indications	Coarctation segment length (mm)	Extent of collateral circulation	Associated cardiac anomalies	Procedure performed
Case 1	39	Male	General discomfort and coldness in both legs, history of CoA repair and VSD closure 29 years earlier.	Recurrent CoA with severe adhesions, hemodynamically significant gradient (>50 mmHg)	7.46	Brachiocephalic artery to the right common iliac artery	N/A	Extra-anatomic ascending-to-descending aortic bypass
Case 2	48	Male	Shortness of breath during exercise and while lying supine, coldness in both legs, history of uncontrolled hypertension.	Significant anatomical CoA with symptoms, hemodynamically significant gradient (>50 mmHg), end-organ impact of hypertension (LVH with diastolic dysfunction)	4.81	N/A	Ascending aortic aneurysm, severe aortic stenosis	Bentall procedure with total arch replacement, repair of coarctatio aorta
Case 3	25	Male	Shortness of breath and coldness in both legs, history of uncontrolled hypertension	CoA with significant pressure gradient (>50 mmHg), associated severe aortic regurgitation	5	N/A	Severe aortic valve insufficiency	Aortoplasty and aortic valve repair

CoA = Coarctation of Aorta, VSD = ventricular septal defect, LVH = left ventricular hypertrophy, N/A = not available.

2. Case series

We report three patients who underwent surgical correction of CoA at our institution between 2018 and 2020. This case series is reported in accordance with the PROCESS guideline [11].

3. Case 1

A 39-year-old male presented with general discomfort and coldness in both legs. He had a history of CoA repair using the patch aortoplasty technique and closure of a ventricular septal defect (VSD) 29 years earlier. Physical examination showed a weaker femoral pulse compared to the radial pulse. Blood pressure in the left arm was higher than in the left leg, with a radio-femoral gradient exceeding 50 mmHg. ECG results were normal. Chest X-ray showed cardiomegaly. Echocardiography revealed residual CoA without residual VSD, left ventricular ejection fraction (LVEF) was 81.7 %, with no other abnormalities (Fig. 1). CT angiography demonstrated CoA measuring 7.46 mm in length distal to the left subclavian artery and proximal to the descending aorta, along with significant adhesions and a Gore-Tex patch on the aorta. The patient was diagnosed with recurrent CoA with severe adhesions. A bypass was performed from the ascending aorta to the proximal descending aorta using an 18 mm Vascutek Gelweave™ graft (Fig. 2a, b). The procedure was successful, and the patient was transferred to the ICU with a radio-femoral arterial blood pressure (ABP) gradient of less than 20 mmHg. Postoperative CT angiography showed well-positioned graft from ascending aorta to descending aorta, with a diameter of 20.3 mm and there is no sign of contrast extravasation, aneurysm, pseudoaneurysm, dissection or wall disruption (Fig. 2c, d). The patient was stable and discharged 8 days postoperatively.

Fig. 1.

Preoperative echocardiography showing residual CoA without residual VSD.

Fig. 2.

Preoperative (a, b) and postoperative (c, d) cardiac CT.

Preoperative CT showing 7.46 mm CoA distal to the left subclavian artery and proximal to the descending aorta, along with significant adhesions and a Gore-Tex patch on the aorta. Postoperative CT demonstrating well-positioned bypass graft.

4. Case 2

A 48-year-old male presented with shortness of breath during exercise and while lying supine, along with coldness in both legs. He had a history of uncontrolled hypertension. Physical examination revealed a weaker femoral pulse compared to the radial pulse. Blood pressure in the right arm was higher than in the right leg, with a radio-femoral gradient exceeding 50 mmHg. ECG showed sinus rhythm, left axis deviation, and left ventricular hypertrophy (LVH). Chest X-ray revealed cardiomegaly, hypertensive heart disease (HHD) configuration, and aortic elongation. Echocardiography demonstrated an ascending aortic aneurysm, severe aortic stenosis, mild aortic regurgitation, LVEF of 61 %, eccentric LVH with diastolic dysfunction (grade II), and normal right ventricular contractility (TAPSE 2.34 cm) (Fig. 3). CT scan revealed an ascending aortic aneurysm (root 60 mm; ascending aorta 70 mm), proximal arch (20 mm), proximal descending aorta (1–2 cm), and descending aorta (25 mm). Additional findings included bicuspid aortic valve and aortic calcification (Fig. 4). The patient was diagnosed with proximal descending CoA, ascending aortic aneurysm, and severe aortic stenosis. A Bentall procedure was performed using a 20 mm Vascutek Gelweave™ graft to replace the aneurysmal section and a 23 mm St. Jude valve for aortic valve replacement. Postoperative echocardiography demonstrated the presence of a conduit in the ascending aorta and confirmed successful replacement of the aortic valve with mechanical prosthesis. The prosthetic valve is well-seated and opens well. No sign of leakage was observed. The radio-femoral ABP gradient was measured at less than 20 mmHg, indicating satisfactory hemodynamic performance (Fig. 5). The patient was discharged 10 days after surgery.

Fig. 3.

Preoperative echocardiography showing ascending aortic aneurysm, severe aortic stenosis, mild aortic regurgitation, LVEF of 61 %, eccentric LVH with diastolic dysfunction.

Fig. 4.

Preoperative cardiac CT showing 4.81 mm CoA with ascending aortic aneurysm (root: 60 mm, ascending aorta: 70 mm), proximal arch (20 mm), proximal descending aorta (1–2 cm), and descending aorta (25 mm).

Fig. 5.

Postoperative echocardiography showing there is a conduit at ascending aorta.

5. Case 3

A 25-year-old male presented with shortness of breath and coldness in both legs. He had been diagnosed with CoA 5 years earlier and had a history of uncontrolled hypertension. The patient sought treatment after worsening symptoms, particularly during physical activity. Physical examination revealed a weaker femoral pulse compared to the radial pulse. Blood pressure in the left arm was higher than in the left leg, with a pressure difference exceeding 50 mmHg. ECG and chest X-ray results were normal. Echocardiography showed CoA and severe aortic regurgitation caused by a bicuspid aortic valve (Fig. 6). Cardiac CT revealed CoA located 30 mm distal to the left subclavian artery, with a 5 mm stenotic segment and proximal intimal hyperplasia (Fig. 7). The patient was diagnosed with left subclavian artery CoA and severe aortic valve insufficiency. The patient underwent aortoplasty with a Vascutek patch and aortic valve repair using a St. Jude mechanical valve (size 19). The patient was discharged 6 days postoperatively, with no significant complaints aside from mild cough (Table 2).

Fig. 6.

Preoperative echocardiography showing CoA and severe aortic regurgitation caused by a bicuspid aortic valve.

Fig. 7.

Preoperative cardiac CT showing CoA located 30 mm distal to the left subclavian artery, with a 5 mm stenotic segment and proximal intimal hyperplasia.

Table 2.

Preoperative echocardiographic results.

Parameters	Case 1	Case 2	Case 3
Aortic root diameter (mm)	31	41	31
Ascending aorta diameter (mm)	25	71	N/A
Descending aorta diameter (mm)	20	27	21.3
Abdominal aorta diameter (mm)	N/A	20	N/A
Peak gradient at CoA site (mmHg)	75	83 (AV PG)	63
Peak velocity at CoA site (m/s)	4.3	4.6	N/A
LVEF (%)	81.7	61.3	64
LVEDD (mm)	54.5	57.4	54.8
LV mass index (g/m2)	105	208	159
LV hypertrophy	Eccentric	Eccentric	Eccentric
LV diastolic function	Normal	Abnormal	Normal
Aortic valve status	Trace regurgitation	Severe stenosis, mild regurgitation	Severe regurgitation

CoA = coarctation of aorta, LV = left ventricle, LVEF = left ventricular ejection fraction, LVEDD = left ventricular end-diastolic diameter, AV PG = aortic valve pressure gradient, N/A = not available.

6. Discussion

According to the 2020 European Society of Cardiology (ESC) guidelines and the 2018 American Heart Association/American College of Cardiology (AHA/ACC) guidelines, surgical intervention in adults with congenital heart disease, specifically CoA, requires individualized assessment considering anatomical details such as the coarctation segment length, extent of collateral circulation and the surgeon's experience [8]. The decision to operate is primarily indicated by significant pressure gradients exceeding 20 mmHg, refractory hypertension and clinical symptoms such as limb ischemia or heart failure. In the present case series, different open surgical techniques were employed tailored to patient-specific anatomy and associated cardiac pathologies [9,10].

Surgical repair of CoA in adults is associated with higher rates of hospitalization and long-term cardiovascular mortality compared to pediatric populations. Despite advancements in surgical techniques, there remains a lack of consensus regarding the most appropriate treatment approach, especially when deciding between a single-stage or two-stage surgical strategy [12]. While the single-stage method can occasionally be completed through a single operation, it often requires more intricate surgical techniques due to the complexity of addressing both the CoA and any associated pathologies in one procedure. In contrast, the two-stage approach involves two separate procedures, typically through a median sternotomy and a posterolateral thoracotomy, which can allow for more focused management of each component but necessitates a longer overall recovery time and increased risk of complications from multiple interventions. Several surgical techniques are available, such as end-to-end anastomosis, extended end-to-end anastomosis, subclavian artery flap aortoplasty, patch angioplasty, interposition tube graft, and extra-anatomical aortic bypass graft (EABG) [13].

All three patients underwent single-stage repair. The patients were positioned in supine, and a median sternotomy was performed for exposure of the thoracic aorta. Subsequently, right atrial cannulation was performed, and cardiopulmonary bypass (CPB) was initiated.

In case 1, the patient had previously undergone CoA repair with Gore-Tex patch aortoplasty 29 years prior. He was now diagnosed with recurrent CoA accompanied by severe adhesions. Recurrent coarctation (re-CoA) refers to the restenosis of the aorta near the original site following an initially successful correction of native coarctation [14]. The incidence of re-CoA has been reported in different studies to be between 5 %–24 % [14,15]. The indications for intervention are similar to those for the primary lesion, including uncontrolled hypertension, a peak pressure gradient exceeding 20 mmHg, and the presence of collateral vessels on imaging studies. Re-CoA has been linked with age at intervention, isthmus hypoplasia, and coarctation diameter. Younger age at the time of intervention, particularly in neonates (<30 days) and to a lesser extent infants under one year is associated with a higher risk of restenosis, especially following balloon dilatation. Restenosis is more likely when the pre-dilatation diameter of the narrowed segment is less than 3.5 mm and post-dilatation remains below 6 mm. Early surgical repair has also been implicated as a risk factor, though some studies suggest that delaying repair may negatively impact long-term survival [14]. In this patient, a single-stage re-repair was performed using an extra-anatomic ascending-to-descending aortic bypass through a median sternotomy. This approach is often preferred in cases of re-CoA with dense adhesions, as it reduces the risk of catastrophic bleeding during re-entry and provides adequate exposure for graft placement [14,16,17]. Intraoperative monitoring included placement of arterial lines in both radial and femoral arteries to assess real-time pressure gradients across the aortic arch, ensuring that the residual gradient remained below 20 mmHg.

In case 2, the patient was found to have multiple aortic pathologies, including proximal descending CoA, ascending aortic aneurysm, and severe aortic stenosis. Surgical intervention for aortic aneurysm involving the arch is typically recommended when the diameter reaches or exceeds 55 mm [18]. Given the extent of the pathology, a single-stage Bentall procedure was performed using a 20 mm Vascutek Gelweave graft to replace the aneurysmal portion, followed by aortic valve replacement with a 23 mm St. Jude Medical mechanical valve prosthesis, which was considered effective. In adult patients, CoA with aneurysmal formations poses significant surgical challenges. Bentall procedure is an uncommon approach for coarctation aorta case alone, but can be used in cases of coarctation of the aorta associated with aortic root problem or proximal aneurysm. This procedure might be beneficial to reduce the need for staged surgeries and minimize the risks of complication [19,20]. Aortic coarctation repair by Bentall and hemiarch replacement can develop aneurysm in the aortic arch distal in decades later. Thus, patient who have undergone coarctation surgical repair require lifelong monitoring to detect late complications that may necessitate further surgical intervention [21].

In case 3, the patient was diagnosed with coarctation of the descending aorta and severe aortic regurgitation. This case presents unique surgical challenges due to the chronicity of the lesion and associated cardiovascular remodeling. A single-stage surgical approach was chosen to address both lesion simultaneously, and patch aortoplasty was selected due to its benefits in enlarging the aortic segment while minimizing total ischemic time to just 10 min in infants and 12 min in adults [22]. This technique can be performed in patients with severe cardiac disease to prevent adverse effect from prolonged cardiopulmonary bypass and aortic cross-clamping time thereby reducing the incidence of complications associated with coarctation repair [23]. Kaya et al. have reported that this technique can effectively reduce the trans-coarctation pressure gradient while enlarging the aortic lumen, often with favorable midterm outcomes [24]. Ultimately, the goal of surgical treatment in CoA extends beyond anatomical correction. It aims to improve long-term cardiovascular function, prevent complications such as hypertension and heart failure, and enhance overall quality of life. In this case, the decision to proceed with single-stage patch aortoplasty allowed for an efficient and effective correction, with the added benefit of avoiding a second operation.

7. Conclusions

This case series underscores the diverse and often complex surgical challenges encountered in adult patients with CoA. Each case required an individualized surgical approach based on the complexity of the coarctation and associated cardiac pathologies. Furthermore, these cases emphasize the critical need for lifelong monitoring, as late complications such as aneurysmal formation or restenosis remain significant concerns. Ultimately, this case series contributes to the growing body of evidence on the management of adult CoA, offering insights into optimal surgical techniques and long-term care strategies to improve patient outcomes.

Author contribution

Tri Wisesa Soetisna: Conceptualization, Project administration, Resources, Formal analysis, Methodology, Supervision, Validation, Writing – Original draft, Review, and Editing; Fegita Beatrix Pajala: Data curation, Formal analysis, Investigation, Methodology, Writing – Original draft, Review, and Editing; Dicky Aligheri Wartono: Conceptualization, Resources, Supervision, Visualization, Writing – Review and Editing; Dudy Arman Hanafy: Conceptualization, Supervision, Writing – Review and Editing; Sugisman: Conceptualization, Supervision, Writing – Review and Editing; Amin Tjubandi: Conceptualization, Supervision, Writing – Review and Editing.

Consent

Written informed consent was obtained from the patient or the patient's parents/legal guardian for the publication of this case series and accompanying images.

Ethical approval

Ethical approval is not required in our institution (National Cardiovascular Center Harapan Kita Jakarta Indonesia) for case reports or case series.

Guarantor

Tri Wisesa Soetisna.

Research registration number

Not applicable.

Funding

The authors report no involvement in the research by the sponsor that could have influenced the outcome of this work.

Conflict of interest statement

The authors certify that there is no conflict of interest with any financial organization regarding the material discussed in the manuscript.