Tricuspid valve prolapse as an early predictor for severe phenotype in children with Marfan syndrome

Stark, V.; Olfe, J.; Pesch, Joerg; Tahir, Enver; Weinrich, Julius Matthias; Wiegand, Peter; Kozlik‐Feldmann, Rainer; Kodolitsch, Yskert Von; Mir, Thomas S.

doi:10.1111/apa.16307

Cited by 7 publications

(4 citation statements)

References 33 publications

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“…In a recent paediatric study of patients with Marfan’s disease, TVP occurred in 68% of the patients and correlated with mitral valve prolapse. 28 Nevertheless, an association between connective tissue disease and TVP is difficult to extrapolate to our cohort because of the significant difference in age, time of disease onset, and co-morbidities between the two cohorts.…”

Section: Discussionmentioning

confidence: 84%

Tricuspid edge-to-edge repair for tricuspid valve prolapse and flail leaflet: feasibility in comparison to patients with secondary tricuspid regurgitation

Dannenberg,

Bartko,

Andreas

et al. 2023

European Heart Journal - Cardiovascular Imaging

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Aims Transcatheter tricuspid edge-to-edge repair (T-TEER) has gained widespread use for the treatment of tricuspid regurgitation in symptomatic patients with high operative risk. Although secondary TR is the most common pathology, some patients exhibit primary or predominantly primary TR. Characterization of patients with these pathologies in the T-TEER context has not been systematically performed. Methods and Results Patients assigned to T-TEER by the interdisciplinary Heart Team were consecutively recruited in two European centers over 4 years. Echocardiographic images were evaluated to distinguish between primary and secondary causes of TR. Both groups were compared concerning procedural results. 339 patients were recruited, 13% with primary TR and 87% with secondary TR. Patients with primary TR had a smaller right ventricle (basal diameter 45 vs. 49 mm, p = 0.004), a better right ventricular function (fractional area change 45 vs. 41%, p = 0.001), a smaller right (28 vs. 34cm², p = 0.021) and left (52 vs. 67 ml/m², p = 0.038) atrium, and a better left ventricular ejection fraction (60 vs. 52%, p = 0.005). The severity of TR was similar in primary and secondary TR at baseline (TR vena contracta width pre-interventional 13 ± 4 vs. 14 ± 5 mm, p = 0.19), and T-TEER significantly reduced TR in both groups (TR vena contracta width post-interventional 4 ± 3 vs. 5 ± 5 mm, p = 0.10). These findings remained stable after propensity score matching. Complications were similar between both groups. Conclusion T-TEER confers equally safe and effective reduction of TR in patients with primary and secondary TR.

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Section: Discussionmentioning

confidence: 84%

Tricuspid edge-to-edge repair for tricuspid valve prolapse and flail leaflet: feasibility in comparison to patients with secondary tricuspid regurgitation

Dannenberg,

Bartko,

Andreas

et al. 2023

European Heart Journal - Cardiovascular Imaging

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“…Also, at follow-up, aortic root dilatation and systemic manifestations were more present in the TVP group than the non-TVP group. Considering that TVP appears early in the disease course, the authors concluded that TVP should be routinely and regularly assessed in the echocardiography of MFS children as a possible predictor of clinical severity outcome (Stark et al, 2022) [51].…”

Section: Valves Tricuspid Valvementioning

confidence: 99%

Unique Features of Cardiovascular Involvement and Progression in Children with Marfan Syndrome Justify Dedicated Multidisciplinary Care

Baban,

Parlapiano,

Cicenia

et al. 2024

JCDD

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Marfan syndrome (MIM: # 154700; MFS) is an autosomal dominant disease representing the most common form of heritable connective tissue disorder. The condition presents variable multiorgan expression, typically involving a triad of cardiovascular, eye, and skeletal manifestations. Other multisystemic features are often underdiagnosed. Moreover, the disease is characterized by age related penetrance. Diagnosis and management of MFS in the adult population are well-described in literature. Few studies are focused on MFS in the pediatric population, making the clinical approach (cardiac and multiorgan) to these cases challenging both in terms of diagnosis and serial follow-up. In this review, we provide an overview of MFS manifestations in children, with extensive revision of major organ involvement (cardiovascular ocular and skeletal). We attempt to shed light on minor aspects of MFS that can have a significant progressive impact on the health of affected children. MFS is an example of a syndrome where an early personalized approach to address a dynamic, genetically determined condition can make a difference in outcome. Applying an early multidisciplinary clinical approach to MFS cases can prevent acute and chronic complications, offer tailored management, and improve the quality of life of patients.

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“…Based on the exclusion of primary congenital anomalies of the TV apparatus, TVP was believed to be secondary to the rupture of chordae tendineae in our case. This lesion has been reported to be caused by ischemic etiologies, including hypoxemia, myocardial infarction, connective tissue diseases, thromboembolism, fibromyxomatous changes, and rhesus isoimmunization, as well as non-ischemic etiologies, such as infectious endocarditis, trauma, endomyocardial biopsy (26)(27)(28)(29)(30)(31). However, it is noteworthy that pressure overload of the RV can also cause rupture of the chordae or papillary muscles.…”

Section: Before Surgery After Surgerymentioning

confidence: 99%

Case Report: unexpected cause of cyanosis in an infant after acute exposure to high altitude—severe tricuspid regurgitation secondary to tricuspid valve prolapse

Cui,

Yang,

et al. 2024

Front. Cardiovasc. Med.

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BackgroundSevere tricuspid regurgitation (TR) causing cyanosis with patent foramen ovale (PFO) and right-to-left atrial shunting requires a precise diagnosis for optimal therapy. Tricuspid valve prolapse (TVP) can lead to TR and is sometimes overlooked, especially in complex cases with factors like pulmonary hypertension (PH). We present an infant with cyanosis and profound TR after high-altitude exposure, initially misattributed to PH but found to be primarily due to spontaneous chordae tendineae rupture and TVP. This case underscores the challenges in diagnosing TR-induced cyanosis.Case presentationThe 3-month-old infant rapidly developed cyanosis, hypoxemia, right atrial enlargement, severe tricuspid regurgitation (TR), and patent foramen ovale (PFO) shunting after high-altitude exposure. Although echocardiography revealed tricuspid valve prolapse (TVP), initial consideration linked TR and right-to-left shunting to pulmonary hypertension (PH) due to the temporal correlation with rapid altitude exposure. Despite hemodynamic stability and the absence of respiratory distress after respiratory support and combined PH medication therapy, the persistent hypoxemia did not reverse as expected. This treatment outcome and repeated echocardiograms reminded us that TR was primarily caused by TVP rather than PH alone. Intraoperative exploration confirmed that TVP was caused by a rupture of TV chordae tendineae and anterior papillary muscle head, and the chordae tendineae/papillary muscle connection was reconstructed. After surgery, this patient was noncyanotic with an excellent long-term prognosis, a trivial TR with normal TV function being observed echocardiographically.ConclusionsTR-induced cyanosis can be not only a consequence of PH and right-sided heart dilation but also a primary condition. Repetitive reassessment should be undertaken with caution, particularly when patients are not improving on therapy in the setting of conditions known to predisposition to secondary TR. Since TVP caused by rupture of the chordae or papillary muscles is rare but fatal in children, early diagnosis is clinically substantial to proper management and satisfactory long-term outcomes.

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Tricuspid valve prolapse as an early predictor for severe phenotype in children with Marfan syndrome

Cited by 7 publications

References 33 publications

Tricuspid edge-to-edge repair for tricuspid valve prolapse and flail leaflet: feasibility in comparison to patients with secondary tricuspid regurgitation

Tricuspid edge-to-edge repair for tricuspid valve prolapse and flail leaflet: feasibility in comparison to patients with secondary tricuspid regurgitation

Unique Features of Cardiovascular Involvement and Progression in Children with Marfan Syndrome Justify Dedicated Multidisciplinary Care

Case Report: unexpected cause of cyanosis in an infant after acute exposure to high altitude—severe tricuspid regurgitation secondary to tricuspid valve prolapse

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