Through the gap: a case series on managing Type B aortic dissection with multiple lumens and tears

ABSTRACT

Aortic dissections are classified as Stanford Type A and B based on site of intimal dissection. Management of Type B dissections is guided by risk stratification. Complicated and high-risk Type B aortic dissections are managed either using endovascular or open surgical repair. Uncomplicated Type B dissections are managed medically. The role of the patency of the false lumen and the presence of reentry tears in the dissecting membrane are still contested. Here, we describe two cases of descending aortic dissections with varying anatomical features in the setting of cocaine use and uncontrolled hypertension. The first case uniquely had a triple lumen dissection with two true lumens, while the second case had two distal tears. Both patients initially had signs of reduced end organ perfusion that resolved with control of comorbid conditions. After multidisciplinary discussions, the decision was made to continue with anti-impulse treatment. Due to the radiological and biochemical absence of evidence of end organ injury even while visceral organs were supplied by the false lumen, our multidisciplinary team preferred conservative management with anti-impulse therapy. This serves as a demonstration of individualized management of Type B aortic dissection in patients with multiple comorbidities using carefully analyzed radiographic and biochemical evidence.

1. Background

Aortic dissection (AD) is the second most common aortic disease in the world with an incidence of about 4.8 cases per 100,000 people per year, after abdominal aortic aneurysms [1,2]. It is also associated with one of the highest mortality rates when compared to other cardiovascular diseases [3,4]. Thus, the incidence may likely be underestimated considering a significant number of patients die prior to hospital presentation [3].

The pathophysiology of acute aortic dissection is attributed to the separation of layers of the aortic wall secondary to a tear in the intimal layer [5]. Subsequent blood accumulation between the tunica intima and tunica media creates true and false lumens with an intimal flap separating the two. Upon development, AD can proceed either anterograde and/or retrograde and can involve the carotid, subclavian, coronary, mesenteric, and renal arteries [6]. Further extension or thrombosis of this false lumen can cause obstruction to the blood flow of the branches of the aorta, resulting in vascular compromise of the end-organs [7]. Several genetic and environmental risk factors can predispose individuals to the development of AD, including hypertension, aortic aneurysms, atherosclerosis, history of aortic surgery, Marfan syndrome, and family history of AD [6,8]. Specifically, conditions that result in a quick and significant rise in blood pressure such as weight lifting and cocaine use have been implicated [6].

There are two major systems for the classification of AD: Stanford and the DeBakey classifications [5]. The Stanford system classifies AD into two categories depending on their origin: ascending proximal to the left subclavian artery called Type A and descending distal to the origin of the left subclavian artery called Type B [5]. Non A-Non B dissections are rarer and arise in the arch of the aorta or in the descending aorta with retrograde extension to the arch [9]. The incidence of Type A aortic dissections (TAAD) surpasses that of Type B aortic dissections (TBAD) and is associated with a significantly higher mortality [1]. Nevertheless, TBAD carries a considerable mortality rate of around 13% in the acute period (<14 days) and up to 30% at 5 years [10,11]. TBAD is considered uncomplicated if there is no evidence of rupture, malperfusion syndromes, or specific high-risk criteria (clinical or radiological) [3].

There has been significant evolution in the management of uncomplicated TBAD, with the focus now primarily on medical management by controlling the heart rate and blood pressure [12]. Aggressive control of these factors decreases the hemodynamic stress on the aortic wall, preventing further progression of the dissection and further complications like rupture or visceral malperfusion which are indications for surgical management [3]. With this “anti-impulse therapy,” the early mortality has been shown to be less than 10%, a rate comparable to early thoracic endovascular aortic repair (TEVAR) [13]. A recent meta-analysis showed that there was similar overall survival in patients who underwent TEVAR compared to optimal medical therapy for acute TBAD [14]. However, late complications of acute TBAD treated medically are not yet fully understood [3]. The INvestigation of STEnt Grafts in Aortic Dissection (INSTEAD trial) evaluated the role of prophylactic TEVAR in uncomplicated TBAD in preventing late complications. Initial results showed that there was no difference in all cause or aorta related mortality at 2 years, but with favorable aortic remodeling occurring in the TEVAR group [15]. However, additional post hoc follow up (INSTEAD-XL) showed that there is better aorta specific survival and delayed disease progression at 5 years [10]. The Acute Dissection: Stent Graft or Best Medical Therapy (ADSORB) trial compared TEVAR+OMT to OMT only and showed increased false lumen thrombosis with combined therapy, but was underpowered to detect a survival advantage [16]. Upcoming clinical trials like Scandinavian trial of uncomplicated aortic dissection therapy (SUNDAY) trial, the IMPRoving outcomes in vascular DisEase – aortic dissection (IMPROVE-AD) trial in the United States, and the Early aortic repair in patients needing endovascular surgery for type B aortic dissection (EARNEST) trial in the United Kingdom aim to provide convincing evidence of the impact of TEVAR in uncomplicated TBAD [17]. Considering the natural history of TBAD, medical therapy may not prevent the eventual thickening of the dissection flap, and therefore could be the pathophysiological reason for continued false lumen perfusion, and thereby worse late outcomes [3]. Aortic fenestration surgeries have been studied as an additional technique in the management of complicated TBAD [3]. When no previous fenestrations naturally occur in the dissection membrane, surgical creation of these fenestrations may be used to connect the true and false lumens to assist with decreasing malperfusion. Computational flow dynamics (CFD) studies have shown that reentry tears result in the return of a portion of blood from the false lumen to the true lumen [18]. This can theoretically create a hemodynamic equilibrium, reducing the pressure on the false lumen, and preventing further aneurysmal degeneration [19]. Norton et al. recently described the use of fenestration surgeries with or without branch stenting in complicated TBAD with low early mortality and excellent long term outcomes [20]. CFD analysis investigating the impact of an additional reentry tear above the distal tear showed that this causes decreased false lumen pressure at the distal end and reduces the risk of false lumen expansion compared to traditional single reentry tears [21]. In swine models, 4D flow MRI has shown that creation of additional reentry tears reduces the false lumen pressure, and increases flow through the true lumen [22]. Clinical studies have shown that having a single entry tear with no reentry tears are a significant predictor for aortic growth compared to a reentry tear, with no further benefit seen with more tears [23]. Tolenaar et al. showed that reentry tears are a significant negative predictor for aortic dilation in patients with TBAD [24]. Additionally, while the impact of multiple distal tears on false lumen thrombosis in patients treated with TEVAR has been investigated, the importance of multiple distal reentry tears in acute uncomplicated TBAD in determining management has not been described in detail [25].

Other anatomical variants with multiple entry tears have also been described in the context of triple-barreled or triple-channeled aortic dissection [26]. They are typically associated with an increased risk of mortality and malperfusion due to compression of true lumen on both sides [26]. However, there is no clear consensus on whether the presence of this triple channeled dissection is enough to call an otherwise uncomplicated TBAD a complicated one [27]. These cases are typically described with having two false lumens and one true lumen, where the third lumen is formed as a result of an acute on chronic pattern especially in patients with Marfan syndrome [28,29]. Only three cases with two true lumens and one false lumen have been identified so far [27,29]. The impact of two true lumens on hemodynamic stability as well as aneurysmal dilation are also not clear, compared to triple channeled aortic dissection in true-false-false configuration where 4D flow MRI has been used [30].

In this context, considering the importance in gaining a better understanding of the various anatomical variations including multiple reentry tears and triple channeled dissections with two true lumens, we present our cases.

2. Case 1 presentation

A 70-year-old male with human immunodeficiency virus (HIV), hypertension, benign prostatic hyperplasia (BPH), and polysubstance abuse presented to the hospital with generalized weakness and new onset crampy back pain.

2.1. Initial diagnosis and assessment

The patient was hypertensive up to 202/91 mm Hg and diaphoretic on initial assessment. He was not tachycardic, and the auscultation revealed a loud S4. His abdomen was soft and non-tender. Peripheral pulses were intact and felt equally bilaterally. Laboratory data were notable for a serum creatinine of 1.7 mg/dL from a baseline of 0.7 mg/dL (reference range <1.3 mg/dL). Cardiac Troponin T was normal at 0.01 ug/L (reference range <0.09 ug/L). Urine toxicology testing was positive for cocaine. An electrocardiogram (EKG) showed normal sinus rhythm and left ventricular hypertrophy. A CT angiogram of the thorax and abdomen showed a Type B aortic dissection starting distal to the left subclavian takeoff with mild dilatation of the descending aorta to 4.6 cm. The dissection extended through the abdomen and pelvis terminating at the level of the left common iliac artery. In the thorax, the dissection showed a second true lumen within the initial true lumen, with fenestrations both proximally and distally (Figure 1(a,b,c,d)). The dissection extended into the celiac and superior mesenteric arteries, however with distal reconstitution of contrast noted (Figure 1(e)). The inferior mesenteric artery was straddling the dissection flap but was also noted to be patent. The right renal artery was supplied by the true lumen while the left renal artery was supplied by the false lumen. A distended urinary bladder with bilateral ureteral dilation and pronounced hydronephrosis was noted as well.

Figure 1.

Case 1 - (a) Sagittal reconstruction of CT angiogram demonstrating both proximal tear (blue arrow) and distal tear (red arrow) within the additional dissection flap located within the true lumen. (b) Axial CT image demonstrates the tear in the true lumen flap (red arrow) (c) Axial CT image demonstrates the more proximal tear in the true lumen flap (red arrow). (d) Axial CT image demonstrates the false lumen (red arrow) and adjacent two true lumens (blue arrows) in a typical “Mercedes Benz” sign pattern. (e) Axial CT image demonstrates the intimal flap of the dissection extending into the superior mesenteric artery (red arrow).

CT: Computerized Tomography.

2.2. Management

Considering the development of TBAD, vascular surgery was immediately consulted and the patient was admitted to the intensive care unit. He was started on a nicardipine infusion to achieve control of systolic blood pressure below 120 mmHg. Urinary catheterization was performed with significant urine output and resolution of back pain. Further labs showed an improvement in renal function back to baseline. Considering that the initial aortic diameter exceeded 40 mm, the patient was considered to be high-risk TBAD and considered for endovascular intervention. However, after multidisciplinary discussion with vascular surgery, critical care medicine, and nephrology, the decision was made to continue conservative management with impulse control therapy. The improvement of renal function, intact peripheral pulses, distal reconstitution of flow in abdominal arteries, and lack of biochemical markers of tissue ischemia were considered in making the decision. The presence of an additional true lumen within the initial true lumen gave a further possible pathophysiological basis for favorable outcomes compared to a true-false-false configuration (Table 1).

Table 1.

Case 1 timeline- 70-year-old male with triple lumen type B aortic dissection.

Day	Event
0	Patient presents with weakness and new-onset back pain. Blood pressure is 202/91 mmHg. CT angiogram shows triple lumen Type B aortic dissection. Labs reveal acute kidney injury. Drug screen with cocaine.
1	Admitted to Cardiac ICU and started on nicardipine for blood pressure control. Urinary catheterization improves back pain and kidney function.
2–3	Multidisciplinary team decides on conservative management. Stable blood pressure and kidney function.
4–5	Discharged with vascular surgery follow-up.
1 Year Later	Re-admitted for unrelated upper respiratory symptoms. No follow-up CT. No recurrent dissection symptoms.

CT: Computed Angiography, ICU: Intensive Care Unit.

2.3. Outcome

After adequate control of blood pressure, he was transitioned to oral metoprolol, hydralazine, and nifedipine. He was counseled on medication compliance and use of cocaine. He was discharged with close follow-up with vascular surgery at an outside hospital but did not appear for the appointments. He was re-admitted 1 year later with unrelated respiratory symptoms and was discharged after a brief stay, however no follow-up CT scans were performed. He did not describe any recurrent symptoms related to prior dissection in the previous year.

3. Case 2 presentation

A 57-year-old male with hypertension, stage 3b chronic kidney disease, and active polysubstance abuse presented to the emergency room with altered mental status, abdominal pain, and vomiting for a few hours preceding the time of presentation. He was recently admitted to an outside hospital with a newly diagnosed intramural hematoma. His Computed Tomography (CT) Angiogram of the thorax and abdomen from the outside hospital is shown in Figure 2(a).

Figure 2.

Case 2 - (a) Transverse CT angiogram of the chest demonstrating intramural hematoma in the descending aorta (yellow arrow) (b) Transverse CT angiogram showing the gap in the dissection membrane with no difference in opacity of contrast through the both lumens. The yellow arrows denote the flaps forming the proximal end of the first tear. (c) Coronal CT angiogram demonstrating the large tears in the dissection membrane. Yellow arrows show the proximal portion of the first tear’s flap. Blue arrow shows the proximal tear in the dissecting flap. Purple arrow shows the intact dissecting flap. Red arrows show the distal tear with mixing of flow through the false and true lumen.

FL: False Lumen, TL: True Lumen CT: Computed Tomography.

3.1. Initial diagnosis and assessment

The patient was hypertensive and tachycardic on initial assessment, with a blood pressure around 150/100 mmHg and a heart rate of 104 beats per minute. His peripheral pulses were intact, and he did not have any murmurs on cardiac exam. While his hemoglobin was at his baseline value, his serum creatinine was elevated at 4.1 mg/dL from a prior value of 1.5 mg/dL (reference range <1.3 mg/dL), consistent with the development of an acute kidney injury. Importantly, urine toxicology testing was positive for amphetamines, cocaine, and opiates. An EKG showed new T-wave inversions in the inferior and lateral leads with elevations of cardiac troponin T to 0.10 ug/L (reference range: <0.090 ug/L). A CT angiogram of the thorax and abdomen revealed a Type B aortic dissection starting distal to the takeoff of the left subclavian artery to the level of the left renal artery. There was no involvement of his aortic arch vessels. The celiac, superior mesenteric, and right renal arteries were supplied by the true lumen, while the left renal artery was supplied by the false lumen. Importantly, there were large naturally occurring reentry tears in the middle and at the caudal ends of the intimal flap with communication of the true and false lumens (Figure 2(b,c)).

3.2. Management

The patient was admitted to the cardiac intensive care unit and started on anti-impulse therapy with an esmolol infusion to reduce systolic blood pressure to <120 mmHg and heart rate <70 beats/min. This resulted in effective pain control. Adequate control of his hypertension resulted in resolution of the acute kidney injury. Resolution of acute drug intoxication over 24 hours resulted in rapid improvement in the patient’s mental status. Renal artery duplex showed patent renal arteries with preserved renal blood flow. A multidisciplinary discussion was held between vascular surgery, critical care medicine, and the cardiology teams. Considering that the contrast density within the bilateral renal and intestinal arteries was similar to the true lumen of the aorta, there was a lack of thrombosis or severe obstruction to these vessels. Coupled with clinical parameters, the multiple reentry tears were thought to have prevented malperfusion of visceral organs. Therefore, neither endovascular repair nor fenestration surgery was pursued (Table 2).

Table 2.

Case 2 timeline-57-year-old male with type B aortic dissection.

Day	Event
0	Patient presents with altered mental status, abdominal pain, and vomiting. Blood pressure 150/100 mmHg, heart rate 104 bpm. CT angiogram confirms Type B aortic dissection. Acute kidney injury (Cr 4.1 mg/dL). Drug screen positive for amphetamines, cocaine, and opiates.
1	Admitted to Cardiac ICU and Started on esmolol for blood pressure control. Kidney function and pain improve. Mental status normalizes as drug effects wear off.
2	Renal artery duplex confirms good blood flow despite false lumen supply. Multidisciplinary team decides on conservative management.
4	Repeat CT angiogram shows no worsening. Blood pressure well controlled, AKI resolves.
5	Transitioned to oral medications and transferred to general medicine floor.

CT: Computed Angiography, ICU: Intensive Care Unit, AKI: Acute Kidney Injury.

3.3. Outcome

A repeat CT angiogram of the abdomen and pelvis was performed 4 days later with no further extension of the aortic dissection. Once his blood pressure was well controlled, he was transitioned to a stable oral antihypertensive regimen and transferred to the general medicine floor.

4. Discussion

Type A aortic dissections require immediate open surgical management due to life threatening complications such as cardiac tamponade from aortic rupture into the pericardial space, bleeding, severe aortic insufficiency, branch obstruction causing strokes, and aneurysmal rupture [3,5]. However, current guidelines recommend further stratification of TBAD into complicated-, high risk-, and uncomplicated TBAD to guide management [3]. TBAD is termed complicated when it causes aortic rupture or malperfusion syndrome. Around 20% of acute TBAD will have malperfusion syndrome. The pathophysiology of malperfusion is either through dynamic forces causing compression of the true lumen branch due to pressure in the false lumen during systole or via static mechanisms where the intimal flap extends into branch vessels and subsequently thrombose. Often, it is a combination of both dynamic and static mechanisms [3]. High-risk features include: refractory pain, refractory hypertension, bloody pleural effusion, as well as certain imaging findings such as an aortic diameter of >40 mm and a false lumen diameter of >22 mm. Both complicated and high-risk dissections are treated with open or endovascular repair [3,11,31]. Uncomplicated TBAD has none of the above features and is managed conservatively with medications in the absence of worsening symptoms or development of complications [3].

Initial medical management of TBAD is aimed at reducing the “impulse force” or the maximum change in left ventricular pressure during the late systole (dp/dt) and is termed anti-impulse therapy [3]. This impulse force is responsible for the development and further propagation of aortic dissections. This is achieved by maintaining BP below 120/80 mmHg and HR below 70 bpm with the help of alpha and beta blocking agents [3,32].

The decision to perform endovascular intervention in a patient with an acute uncomplicated TBAD is not simple and has been heavily contested over the last two decades. Prior clinical trials like INSTEAD, INSTEAD-XL, and ADSORB as well as upcoming trials evaluating this have been discussed previously. In some observational studies, 46% to 58% of the patients with TBAD failed medical therapy and required intervention [33,34]. However, other observational studies have shown excellent survival rates in patient with TBAD treated medically [35–37]. A recent meta-analysis by Hossack et al. evaluating 14,706 patients showed no difference in inpatient mortality in the acute period of TBAD when comparing medical therapy to TEVAR (RD, 0.01; p = 0.46) [13]. Therefore, while there may not be significant differences in early mortality, the impact of early prophylactic TEVAR on late outcomes needs further investigation.

In our second case, once there was resolution of severe hypertension causing the AKI, the case could be managed as an uncomplicated TBAD. The multiple reentry tears and the resulting patent false lumen add additional complexity to the decision-making that is not described in detail in recent guidelines [3]. Data on using the patency of false lumen to determine the severity of dissection and to decide on endovascular repair are heterogeneous. Li et al. showed that in patients with TBAD, the presence of a patent false lumen was associated with an increased risk of mortality (HR, 2.12; p < 0.001) and is associated with >7-fold increase in aortic events, compared with a fully thrombosed FL [38]. At the same time, a more recent meta-analysis by Romeiro et al. showed that having a patent false lumen did not increase the risk of complications in those initially diagnosed with uncomplicated TBAD (HR 1.13; 95% CI: 0.79–1.60) [39]. Other reports have also found a patent FL to result in a better prognosis than having a partially thrombosed false lumen [38,40].

The size and number of fenestrations or tears in the dissection membrane may play a role in predicting aneurysmal dilation [3]. As noted previously, CFD and 4D Flow MRI studies have shown that having an additional tear above the distal reentry tear helps with preventing false lumen expansion [21,22]. In a study by Kotelis et al. evaluating chronic TBAD, having >2 reentry tears was associated with increasing aortic dilation [41]. They argue that both patients with no distal reentry tears and those with two or more distal reentry tears are at higher risk for aortic dilation and poor later outcomes, while those with one entry and one reentry tear could be managed conservatively [41]. Therefore, the presence of an additional reentry tear in our second case makes for an additional level of complexity in decision-making regarding early TEVAR. Our case suggests that in the acute phase of TBAD, the presence of multiple reentry tears protects against thrombosis of the false lumen and preserves blood flow to visceral arteries supplied by the false lumen preventing malperfusion syndromes. The decision regarding TEVAR can then be delayed to the subacute period, based on subsequent imaging and clinical follow-up, since early TEVAR is associated with an increased risk of peri-procedural complications [3]. Therefore, in patients with multiple distal reentry tears in an otherwise uncomplicated TBAD, early conservative management with anti-impulse therapy followed by referral for early TEVAR in the sub-acute period may be required to prevent late complications.

The presence of three lumens or channels in the dissection is rare and is usually associated with patients who have Marfan syndrome [42]. The typical CT finding in such patients is the “Mercedes Benz” sign with a balanced three lumen areas [43]. This was demonstrated in our patient in case 1 as well (Figure B). Recurrence of pain in patients with established AD has been shown to be an important indicator for the development of triple channel dissection [29]. The role of triple channel dissection in optimal treatment strategies or outcomes is currently not well described in the literature [42]. Both the Society for Vascular Surgery/Society of Thoracic Surgeons and the American Heart Association guidelines for management of TBAD do not include the development of multi-channel dissections as a risk factor [3,5]. In contrast to our patient in case 1, almost all triple channel dissections that have been described in the literature are in a true-false-false configuration, with the new lumen developing in the old false lumen [27]. This is associated with increased mortality, and the risk for rapid expansion and rupture due to added stress on a weak false lumen wall [27]. There have also been quadruple lumen dissections described in the literature; however, additional lumens were also formed inside the initial false lumen [44]. In an analysis of 31 patients who underwent surgical management for triple lumen aortic dissections, three triple lumen dissections were described to have developed in the media of the true lumen [29]. However, the study included both Type A and B aortic dissections, and data are not available on whether the dissections with a second true lumen occurred in TBAD [29]. Additionally, specific management and outcomes for these patients have not been described [29]. While we are limited by the description of a single case, the management of the patient in case 1 serves as important data-point in the world of true-true-false configuration of TBAD triple lumen dissections. There was no clinical recurrence of chest pain or laboratory evidence of malperfusion at an incidental admission 1 year later. There needs to be further investigation and studies to analyze whether the increased mortality associated with triple channeled TBAD extends to those with two true lumens as well.

In our patients, the distal reentry tears in the dissection membrane allowed blood flow through both lumens without thrombosis or expansion. There was no suggestion of dynamic or static obstruction in the renal arteries and the mesenteric arteries radiographically. Additionally, our patients lacked any laboratory findings suggestive of tissue ischemia. In the arterial phase, there did not seem to be any significant difference in intensity of contrast between the two lumens, denoting lack of thrombosis of the false lumen. The absence of clinical malperfusion symptoms and radiological support allowed our cardiology, vascular surgery, and radiology teams to manage these patients medically in the hospital.

Our study therefore describes two entities that have not been described in great detail in guidelines. While some cases of multiple reentry tears have been described in case reports and reviews, our cases attempt to explore the decision-making regarding the need for early or late endovascular intervention in detail. The case with two true lumens is the first that has been described in detail with imaging evidence. All prior case reports with triple or multi-lumen aortic dissections have been in patients with a true-false-false configuration. While patients with a true-false-false lumen may be at higher risk for complications because the second false lumen is in the weak media of the first, the same may not be the case in a true-true-false configuration. Our study also attempts to display the importance of estimating the risk of TEVAR in patients with polysubstance use, who may not be adherent to perioperative antiplatelet use which may cause further complications down the line. In this manner, we aim to explore the importance of these two anatomical variants of TBAD and its impact on management decision-making.

Our study is limited primarily by its design since while our descriptions of individual cases shed light on important disease phenotypes, conclusive evidence regarding the superiority of one management approach over another cannot be made. Additionally, due to the limited imaging follow-up of our patients, sub-clinical changes in aorta anatomy may have been missed. Advanced studies like 4D flow MRI were also not performed in these cases. However, we want to use these cases to highlight the intricacies in the management of uncomplicated TBAD, especially to prevent procedural complications in the acute period, and prevent late aortic dilation-related complications in the chronic period. Additional registry-based observational studies and eventually randomized controlled trials need to be performed to determine the ideal timing of TEVAR in patients with multiple distal tears and to determine if OMT has superior outcomes than TEVAR in patients with true-true-false triple channel TBAD compared to those with two false lumens.

5. Conclusion

The management of uncomplicated TBAD is challenging, especially in patients with multiple comorbidities. In patients with TBAD and malperfusion, artificial fenestration, both surgical and endovascular have been described as safe and effective methods of management. The cases provided show that anatomical variations, including the number of distal reentry tears, as well as the number of lumens, may require additional consideration for both the type and timing of definitive management in patients with TBAD with high-risk features. Larger studies are needed before conclusive evidence can be found regarding the management of this subgroup of patients.

Funding Statement

This paper was not funded.

Article highlights

• Management of Type B Aortic Dissection (TBAD) depends on the presence of complications like rupture or malperfusion syndromes to determine if Thoracic Endovascular Aortic Repair (TEVAR) is required.

• Uncomplicated Type B Aortic Dissections are typically managed with anti-impulse therapy, however, The INvestigation of STEnt Grafts in Aortic Dissection (INSTEAD-XL) and The Acute Dissection: Stent Graft or Best Medical Therapy (ADSORB) trials suggest the importance of early TEVAR in preventing late complications.

• Anatomical variations such as multiple distal reentry tears and triple lumen dissections are rare and its impact of decision-making for definitive management is not well described in current guidelines.

• The first case describes a triple lumen TBAD, in an extremely rare true-true-false configuration. Conservative management was pursued despite an additional high-risk feature and showed good short- and long-term clinical outcomes.

• The second case describes an uncomplicated TBAD with an extra distal reentry tear, and focuses on the impact of additional reentry tears on the need and timing of TEVAR.

• Multiple distal reentry tears may preserve false and true lumen perfusion without causing false lumen expansion, which challenges the traditional view that all patent false lumens are at increased risk of complications.

• Increasing use of computational flow dynamics and 4D Flow Magnetic Resonance Imaging may be needed to better characterize false lumen pressure dynamics and flow patterns which would help refine risk stratification and guide the type and timing of management, especially in anatomically complex cases.

• The management of TBAD with such anatomical variations requires a multi-disciplinary approach with vascular surgery, cardiology, and radiology input to ensure that clinical, anatomical, and radiological data is comprehensively evaluated.

• Additional larger observational studies and clinical trials are required to better understand the ideal management in this subgroup of patients.

Author contributions

S.S.K., S.N., and S.K. worked on the cases included in the manuscript. S.S.K., S.N., N.A., A.B., and S.M.S. participated in making the discussion aspect of the manuscript. M.G. worked as the senior author in the review and analysis of all images included in the manuscript.

Sanjana Nagraj is a member of the Future Cardiology Editorial Board. They were not involved in any editorial decisions related to the publication of this article, and author details were not made available to the article’s peer reviewers as per the journal’s double-anonymized peer review policy.

Disclosure statement

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Ethical declaration

This case series was conducted in compliance with ethical guidelines and institutional policies for the use of patient information in research. Informed consent and/or surrogate consent were obtained as outlined below:

• Case 1: Written informed consent was obtained directly from the patient after a thorough explanation of the case series’ purpose, methods, and potential publication of de-identified clinical details. The patient provided their explicit agreement to participate.

• Case 2: Verbal assent was obtained from the patient and written informed consent was obtained from the patient’s surrogate, who is his legally authorized representative and sister, following a detailed discussion of the case series’ aims and the use of anonymized clinical information.

All identifiable patient information has been removed or anonymized to protect confidentiality.