Tricuspid annular disjunction with varying degrees of tricuspid regurgitation in the setting of Takotsubo syndrome

Abstract

Tricuspid annular disjunction (TAD) is concomitant in approximately half of mitral annular disjunction (MAD) cases. Here we report a case of echocardiographically isolated TAD detected during Takotsubo syndrome (TTS) complicated by a transient aggravation of tricuspid regurgitation. An 87‐year‐old female was admitted at the emergency department with ST segment elevation. Coronary angiography findings were consistent with TTS. Transthoracic echocardiography (TTE) showed a left ventricular apical aneurysm with incidental finding of TAD with ‘torrential’ tricuspid regurgitation. Importantly, no concomitant MAD was detected on TTE. No significant arrhythmias were detected on telemetry surveillance. Follow up TTE showed improvement in left ventricular function with reduced regional wall abnormalities. TAD was still present although the tricuspid regurgitation had reduced to ‘moderate’. The patient was discharged home after 23 days of hospital stay. The present case illustrates the need of further investigations into TAD and its clinical implications for acute TR in TTS.

Introduction

Tricuspid annular disjunction (TAD) is defined as ≥1.0 mm end‐diastolic distance between the tricuspid valve leaflet junction and the basal right ventricular wall. ¹ This structural alteration is observed in 50% of patients with mitral annular disjunction (MAD), suggesting one annulus continuum, as opposed to separate mitral annulus and tricuspid annulus. ¹ At present, there is only one reported case of isolated TAD in the absence of concomitant MAD, documented by means of cardiac magnetic resonance (CMR), which was potentially associated with ventricular arrhythmias. ² TAD is often unrecognised; thus, having this as the first case describing TAD incidentally diagnosed with transthoracic echocardiography (TTE) without concomitant MAD. Similar to signs of MAD on the mitral annulus, a ‘Pickelhaube sign’ was observed with tissue Doppler imaging on the tricuspid annulus in the present case. The latter observation provides suggestion for similar echocardiographic signs of annular disjunction on the left and right side, albeit no echocardiographic TAD diagnostic criteria have been established and the prognostic impacts of TAD remain unknown. The patient experienced Takotsubo syndrome (TTS), which aggravated the degree of the TAD‐associated tricuspid regurgitation (TR).

Case Report

An 87‐year‐old female arrived at the emergency department via ambulance presenting with nausea, repeated vomiting and abdominal pain in the inferior quadrants. Medical history included kidney failure and surgery for vulvar cancer. Upon physical examination, an inguinal hernia was found. No abdominal congestion or other decompensation signs were observed, and no systolic murmur was documented at admittance. A subsequent electrocardiogram showed ST segment elevation most pronounced in leads V2 and V3 (Figure  1 ).

Figure 1.

Electrocardiogram upon hospital admission.

Coronary angiography showed no significant coronary stenoses (Figure 2 A–D ). Left ventricular angiography was remarkable for basal hyperkinesis with mid‐to‐apical hypokinesis and left ventricular ballooning (Video S1), leading to a typical Takotsubo pattern. ³ The patient was admitted to a cardiology ward and troponin T was 311 ng/mL.

Figure 2.

Contrast angiography of the three main coronary arteries: (A) Left main artery in RAO caudal projection, circumflex; (B) apical LAD view; (C): off axis left main artery view; (D): right coronary artery and (E) Coronary physiological measurements of Fractional flow reserve (FFR), coronary flow reserve (CFR), and microcirculatory resistance (IMR) of 68 which indicates microvascular dysfunction.

CMR was not performed at this time due to low glomerular filtration rate (GFR). TTE was performed and showed a dilated left ventricle with apical ‘ballooning’ whilst basal segments displayed hyperdynamic systolic contraction, which were consistent with the angiographic findings (Video S1) and supportive of the TTS diagnosis. ³ Ejection fraction (EF) was 34%. There was a TR, which was primarily mid‐late systolic with eccentric jet directed towards the atrial septum. The PISA radius (Figure 3 A ) measured 13 mm, the effective orifice area (EROA) was 1 cm² and the regurgitant volume (RV) was 99 mL thus grading the TR as ‘torrential’ according to novel grading criteria. ⁴ By method of three‐dimensional vena contracta (Figure 3 B ), the regurgitation is classified as ‘massive’. ⁴

Figure 3.

(A) Two‐dimensional TTE apical four‐chamber image with PISA radius measurement. (B) A three‐dimensional vena contracta measurement using GE® flexi slice analysis. (C) Tissue Doppler showed late systolic peaking of the tricuspid valve S wave which is a characteristic of the known ‘Pickelhaube sign’ often observed with mitral annular disjunction. (D) Linear measurement of the longitudinal displacement of the tricuspid lateral annulus during systole. RA, right atrium; RV, right ventricle. Three‐dimensional image (E) visualising from the atrial side of the tricuspid valve showing pointer to the prolapsed posterior leaflet, with the aortic valve positioned at 5 o'clock. MV, mitral valve; AV, aortic valve; S, septal leaflet; P, posterior leaflet; A, anterior leaflet.

TTE in addition showed a hypermobile lateral tricuspid annulus with ‘curling’ and was labelled as TAD. There were no reciprocal findings on the left sided annulus (Video S2). As per echocardiographic signs of MAD, a Pickelhaube sign (Figure 3 C ) was detected using tissue doppler. The maximal annular displacement was 7 mm (Figure 3 D ). A tricuspid leaflet prolapse was also observed (Figure 3 E ) and the right ventricular systolic pressure (RVSP) was estimated at 60 mmHg.

During the following days the clinical presentation of the patient deteriorated. N‐terminal pro‐brain natriuretic peptide (NT‐proBNP) levels were >35 000 ng/L and troponin T had increased to 643 ng/mL upon the third day of admission. Management was focused on relieving abdominal pain and start heart failure treatment, which was initially complicated by hypotension. In addition, the patient's renal function was markedly impaired with GFR 24 mL/min/1.73 m² but did not require dialysis. A clinical improvement was achieved within a few days with treatment including inotropes, antibiotics, low‐molecular weight heparin, and pain relief as well candesartan initiation. No significant arrhythmias were noted on telemetry up to 7 days when the patient was transferred to a geriatric ward.

A follow up TTE on the 13th day of hospitalisation showed improvement in left ventricular systolic function (EF: 49%) with a reduction in akinetic and aneurysmal segments. A reduction of TR led to a reclassification to ‘moderate’ using PISA formula (EROA: 0.3 cm², RV: 21 mL). RVSP had reduced to 32 mmHg. Importantly, the distance of TAD remained unchanged.

The clinical status successively improved and the patient was discharged home after 23 days of hospital stay.

It is important to note, after analysing images from a TTE performed 4 years before the presentation with TTS, a TAD of similar distance was detected, although it was not noted in the TTE report. The TR severity (EROA: 0.14 cm2; RV: 9 mL) and RSVP (30 mmHg) were similar to the post TTS observations.

Although CMR is not contra‐indicated, no CMR was performed in the acute phase or follow‐up.

Discussion

The present case is the first report of an echocardiographically observed TAD, in the absence of concomitant MAD. During TTS, the TAD‐associated TR was aggravated to torrential, whereas the pre‐ and post‐TTE showed a mild‐ and moderate TR, respectively, with unchanged TAD distance. Finally, the present case observed a Pickelhaube sign on the tricuspid annulus similar to that seen on the mitral annulus with MAD, which leads to the possibility of applying the Pickelhaube sign as a diagnostic criteria on TTE.

Currently, MAD is receiving a lot of attention, whilst TAD is a developing phenomenon. In a study of 84 subjects with MAD, concomitant TAD was present and in 50% of the patients, ¹ which has led to the ‘bi‐annular disjunction’ concept of one annulus continuum rather than separated mitral annulus and tricuspid annulus. ¹ TAD was associated with more severe left sided annulus disjunction. ¹ In contrast to MAD being isolated in 50%, only one reported case has reported an isolated TAD. ²

TAD is primarily diagnosed using CMR and although visible on TTE, ¹ there is presently a lack of TTE diagnostic criteria. The previous case of an isolated TAD was diagnosed using CMR, however, interestingly not seen with TTE. ² The patient in the present case did not undergo CMR due to renal failure and unstable clinical status, and a concomitant MAD cannot be completely excluded. We however noted a Pickelhaube sign on tissue Doppler with late systolic velocities ≥17 cm/s at the tricuspid but not reciprocal findings on the mitral annulus. This observation suggests that MAD was not detected on TTE and that there is a possibility of echocardiographic diagnostic criteria that can be applied to TAD.

Although TAD was detected during TTE as an incidental finding as the current diagnosis was TTS, there appears to be no relationship between the temporary TTS condition and the TAD. TAD was seen without significant change in distance, in retrospect, on a 4 years precedent TTE, as well as on the follow up TTE after TTS regress. In contrast, we observed a temporary worsening of TR during TTS in the present case. When considering treatment protocols for TTS, there is no current additional recommendations relating to the TAD diagnosis as there are no current suggested treatments towards TAD as there was no correlation between TAD and ventricular arrhythmias. ¹ A previous study reported that 25% of patients suffering from TTS had acute mitral regurgitation. ⁵ In contrast, TR is more uncommon and unrelated to right ventricular TTS involvement. ⁶ In the present case with TAD, the tricuspid regurgitation was substantially aggravated during the acute phase of TTS compared with the TTE obtained before and after, as illustrated in Video S3. The TR aggravation may have been related to the increased RVSP in the present case, and the specific relation between TAD and TTS‐induced TR merits further study. Longitudinal strain of the right ventricle (Video S4) showed no segmental reductions of the free wall, thus displaying no confounding evidence of right ventricular involvement in TTS and further strengthening the argument that the aggravated TR is a result of increased RVSP most likely secondary to increased left ventricular pressures.

The present case did not reveal any significant arrhythmias during 7 days telemetry. Concomitant TAD has not been associated with ventricular arrhythmias in MAD, ¹ but the reported case of isolated TAD was concluded to be arrhythmogenic. ² The presence of a Pickelhaube sign in MAD is however associated with arrythmias, ⁷ whereas it is of unknown clinical significance in the present case.

Conclusions

Whilst current research involved CMR diagnosis of TAD, TTE can potentially prove to be helpful as a diagnostic tool in TAD. We show that TAD can be measured on TTE as well as the adaptation of secondary signs such as the Pickelhaube sign as seen with MAD. Whilst there were no observations on TTE to confirm the presence of MAD, there is a need for CMR to exclude the presence of MAD, thus to confirm isolated TAD. Echocardiography can however be useful in the absence of CMR. The visual curling motion and pronounced Pickelhaube sign display good evidence of the presence of TAD though the relationship with TTS remains unexplained This case showed no influence on TAD severity during TTS but a pronounced TR aggravation, likely related to pressure overload of the right ventricle secondary to increased left ventricular pressures. The findings in the present case raised the need for further investigations on echocardiographic imaging of TAD as well as into if the presence of TAD increases the severity of present TR during an incidental Takotsubo event.

Conflict of interest

None declared.

Supporting information

Video S1. Supporting Information

Video S2. Supporting Information

Video S3. Supporting Information

Video S4. Supporting Information

Gaylard B., Montaño‐Krawczuk A., Haugaa K., and Bäck M. (2024) Tricuspid annular disjunction with varying degrees of tricuspid regurgitation in the setting of Takotsubo syndrome, ESC Heart Failure, doi: 10.1002/ehf2.14704