Background: Constrictive pericarditis is a rare inflammatory condition characterized by the thickening and stiffening of the pericardium, leading to impaired cardiac function. Alport syndrome, a hereditary renal disorder, and end-stage renal disease (ESRD) can complicate the clinical course of constrictive pericarditis 

Objective: This study aims to present a case report of constrictive pericarditis in a patient with Alport syndrome and ESRD, highlighting the diagnostic challenges and management strategies. Case report: A 31-year-old male with a known history of Alport syndrome and ESRD, who underwent kidney transplantation that failed, presented with symptoms suggestive of constrictive pericarditis. Diagnostic evaluation revealed findings consistent with constrictive pericarditis, and the patient underwent pericardiectomy, leading to rapid clinical improvement. 

Discussion: The presented case emphasizes the importance of considering constrictive pericarditis in patients with Alport syndrome and ESRD, particularly those with a history of failed kidney transplantation. Early recognition and appropriate management are crucial for optimizing patient outcomes in this complex population. 

Conclusion: Constrictive pericarditis can occur in patients with Alport syndrome and ESRD, posing diagnostic and management challenges. Timely intervention, such as pericardiectomy, can lead to favorable outcomes in these patients.

Constrictive pericarditis is a chronic inflammatory disease of the pericardium that leads to the thickening and stiffening of the pericardial sac, impacting myocardial function, resulting in the inability of the myocardium to fully relax [1,2].

Consequently, due to decreased pericardial compliance, ventricular pressures rapidly rise, leading to decreased cardiac output, manifesting as edema, fatigue, weakness and dyspnea [3]. Ascites, hepatomegaly, dyspnea, and orthopnea are other characteristic symptoms of constrictive pericarditis. While constrictive pericarditis is relatively rare, its diagnosis and management pose significant challenges due to its varied clinical presentation and the potential for mimicking other cardiac and non-cardiac conditions [4]. In this article, we review the pathophysiology, diagnostic modalities, and treatment options for constrictive pericarditis, highlighting the importance of early recognition and intervention in improving patient outcomes. 

As we pointed, the clinical manifestations may sometimes mimic myocardial infarction (MI), aortic dissection, pneumonia, and connective tissue disorders, as well as restrictive cardiomyopathy, amyloidosis, hemochromatosis, and other infiltrative disorders [5]

Treatment typically involves the use of anti-inflammatory medications, and pericardiectomy may be considered as definitive therapy [6]. Therefore, timely diagnosis and treatment of the disease can be life-saving for the patient [7]. Since the diagnosis of this condition is challenging due to its rarity, in this study, we report a case of constrictive pericarditis in a patient with Alport syndrome who underwent kidney transplantation and discuss their association. 

The case involves a 31-year-old male with a known diagnosis of end-stage renal disease (ESRD) and Alport syndrome. He has been undergoing dialysis for the past 8 years and was a candidate for kidney transplantation. However, six months ago, the patient underwent a kidney transplant which was rejected by his body. Over the past two weeks, he has been experiencing episodes of hypotension, dizziness, and severe weakness following each dialysis session. His blood pressure was between 90/60 – 80/55. These symptoms have persisted continuously for two weeks, accompanied by exacerbation of fatigue and occasional mild dyspnea. As a result of these complaints, the patient referred to our hospital. Upon examination, vital signs and SaO2 were within normal limits (SaO2=94%, HR=78, RR=20, T=37.1), and there were no symptoms of fever, cough, or chest pain. Laboratory investigations revealed anemia and metabolic acidosis (Table, 1),

                                                                                                       Table 1: Laboratory Data on Admission.

with mild hepatomegaly noted on physical examination. Besides hypotension, the patient did not complain of any cardiac, vascular, or chest pain symptoms. Given the presentation of mild dyspnea and hypotension, a chest CT scan was performed to investigate for COVID-19, which did not show any signs of lung tissue involvement but reported calcification of the pericardial wall (figure.1). 

                                                                                                        Figure 1: calcification of the pericardial wall

Further evaluation with echocardiography revealed severely abnormal diastolic function, abnormal septal motion, and severe dilatation of the left atrium (LA) and right atrium (RA).  The patient presented with several clinical and diagnostic findings suggestive of constrictive pericarditis. These included episodes of hypotension, dizziness, and severe weakness, which are common manifestations of constrictive pericarditis due to impaired cardiac output [8]. Additionally, he complained of occasional mild dyspnea, likely resulting from impaired diastolic filling due to pericardial constriction. Laboratory investigations revealed anemia and metabolic acidosis, while physical examination showed mild hepatomegaly, both consistent with the diagnosis of constrictive pericarditis. Imaging studies further supported the diagnosis(1), with chest CT scan showing calcification of the pericardial wall and echocardiography demonstrating severely abnormal diastolic function and dilatation of the LA and RA.  Based on these clinical and diagnostic findings, the patient was promptly diagnosed with constrictive pericarditis and referred for further management, including surgical intervention.

The presented case underscores the intricate relationship between end-stage renal disease (ESRD), Alport syndrome, and constrictive pericarditis. Alport syndrome, a hereditary disorder characterized by progressive renal impairment, often culminates in ESRD [9]. This genetic condition arises from mutations in genes encoding type IV collagen, predominantly affecting the glomerular basement membrane [10]. Patients with Alport syndrome frequently necessitate renal replacement therapy, such as dialysis or kidney transplantation, to manage ESRD. In the case described, the patient's history of Alport syndrome and subsequent kidney transplantation, which unfortunately failed and led to kidney rejection, illustrates the complexities in managing ESRD in this population [11]. The failure of the transplanted kidney necessitated a return to dialysis, further complicating the patient's clinical course.  Furthermore, the patient developed symptoms consistent with constrictive pericarditis, a condition characterized by impaired cardiac function due to pericardial thickening and stiffening [12]. According to Kleynberg (2011), constrictive pericarditis can arise from various etiologies, including inflammatory or infectious processes, radiation therapy, or previous cardiac surgery [13], that the presented case hadn't any of them. Although the precise etiology of constrictive pericarditis in this case remains unclear, the patient's history of Alport syndrome and kidney transplantation may have contributed to its development. The diagnostic evaluation revealed findings suggestive of constrictive pericarditis, including episodes of hypotension, dizziness, severe weakness, mild dyspnea, and abnormal echocardiographic findings [14]. Imaging studies further supported the diagnosis, with evidence of calcification of the pericardial wall observed on chest CT scans. Prompt recognition of constrictive pericarditis facilitated timely intervention and referral for pericardiectomy, resulting in the patient's rapid clinical improvement.,In conclusion, this case underscores the importance of considering constrictive pericarditis in patients with ESRD and Alport syndrome, particularly those with a history of failed kidney transplantation. Early recognition and appropriate management of constrictive pericarditis are crucial for optimizing patient outcomes and preventing further complications in this vulnerable population. Further research is warranted to elucidate the underlying mechanisms linking Alport syndrome, ESRD, and constrictive pericarditis, as well as to explore potential therapeutic strategies for these patients.

The authors would like to thank the Clinical Research Development Unit (CRDU) of Emam Ali Hospital, Alborz University of Medical Sciences, Karaj, Iran, for their support, cooperation and assistance throughout the period of study. 

Conflict of interest:

All of authors have no conflict of interest