**5. Epidemiology and etiology**

*Inflammatory Heart Diseases*

tomy for constrictive pericarditis successfully.

**3. Anatomy and function of the pericardium**

ensures heart stabilization and anchoring [1–3].

significantly affect the results after pericardiectomy.

Key observations are described:

stroke volume reduction.

late diastolic period.

**4. Pathophysiology**

a pericardotomy for an effusion in 1649. Then, Vieussens, some years later in 1679 and 1715, observed some cases of pericardial adhesions; from that step, the concept of constrictive pericarditis was born. Afterward, in 1728, Lancisi confirmed the existence of constrictive pericarditis and its risks such as cardiac compression and death via necropsy studies. Those observations were also mentioned by Morgagni (1761), Senac (1749), Laennec (1819), and Cheevers (1842). Clinical patterns related to the right atrium and ventricle compression, mainly as the most important source in the compression process, has been emphasized by Wilkes (1870). Based on anatomical findings, clinical pictures were then clarified: Kussmaul (1873) pointed out the venous pressure elevation in case of constrictive pericarditis; Pick (1896) described a new entity named "Pericarditis pseudocirrhosis of the liver." Since then, Pick Syndrome was recognized as a component of constrictive pericarditis clinical presentation. From that, surgical treatment was considered as the most efficient therapy for releasing heart compression due to pericardium thickness and fibrosis. By then, Rhen and Sauerbruck (1913) in Germany, Hallopeau in France (1921), Schmieden and Fisher (1926) in Germany, Churchill (1929) and Beck (1931) in USA performed the first cases of pericardiec-

The pericardium is a solid fibro-serous sac that maintains the heart in the anterior mediastinum in a closed thoracic cavity called the "pericardial cavity." The pericardium has two main components: (1) an external, fibrous pericardium ensuring the fixity of the pericardium and (2) an internal serous pericardium with two principal layers: the visceral and the parietal pericardium. In a normal heart, the parietal and visceral pericardium release the pericardial cavity filled with a fluid up to 50 ml allowing easy systolic and diastolic heart movements. Pericardium prevents the heart from inflammation, infection, damage, and excessive dilatation; it also

In case of constrictive pericarditis, restricted diastolic heart distensibility does exist and may provoke a right and a left ventricular preload decrease with a reduction of stroke volume and cardiac output [2, 3]. In Africa, we use to face on late clinical presentation of patients with massive pericardial thickness and calcifications inducting myocardial atrophy, fibrosis, and severe systolic dysfunction that

1.Impairment of ventricular distensibility and filling coexisting with ventricular

3.High diastolic plateau due to a small expansion of the ventricular volume at the

All those consequences determine the so called "Dip-and-plateau waveform."

2.Abnormal rapid diastolic ventricular filling and abnormal venous pressure

elevation followed by an early diastolic ventricular dip.

**42**

Classically, constrictive pericarditis appears as a complication of acute or effusive pericarditis. From Yadav's study [4], approximately 9% of patients with acute pericarditis will contract a pericardial constriction. In Africa, the frequency of acute pericarditis varies widely with geographical location ranging from 2 to 11.3% among patients admitted in a hospital for cardiovascular diseases [5–7]; it affects mostly young male population with an average age between 26 and 42 years old and tuberculosis as the most frequent etiology from 33 to 69.5% in sub-Saharan Africa [5, 6, 8]. Over the past decades, the incidence of tuberculous pericarditis has risen up because of the HIV epidemic in sub-Saharan Africa [8, 9]. Noubiap et al. [10] have reported a comprehensive understanding of the epidemiology of pericardial diseases in Africa confirming clearly that tuberculosis remains as the leading cause of pericardial diseases in African Resource-Limited Settings with poor outcome marked by a mortality rate between 18 and 25% including a very high one of 40% within 6 months among patients with HIV/AIDS positive. However, pericardial tuberculosis frequency is variable according to authors such as Mayosi [11] and Thwaites [12] who found, respectively, pericardial tuberculosis in 69.5% of cases admitted for pericardiocentesis and in 10% of all hospitalized patients with heart failure. Moreover, the risk for developing constrictive is very high for tuberculosis or purulent pericarditis: 20–30% and in almost all the cases of tuberculous pericarditis as reported by Gupta [13]. In their prospective study on 500 consecutive cases, Imazzio et al. [14] have demonstrated that the evolution from non-constrictive pericarditis to constriction is different according to pericarditis etiology: the risk of constriction is greater for bacterial etiologies (tuberculosis or purulent pericarditis) than viral or idiopathic acute pericarditis; the incidence rate of constriction is, respectively, 31.65 cases per 1000 person-years for tuberculous pericarditis and 52.74 person-years for purulent pericarditis versus 0.76 person-years for viral or idiopathic pericarditis. The same observation has been described by Permanyer-Miralda et al. [15] in a prospective study of patients with occurrence of constriction in 56 and 35% of patients with tuberculous and purulent pericarditis, respectively, and in 17% of patients with neoplastic pericarditis after an acute pericarditis. In Africa, and in literature overall, the epidemiological pattern, incidence, and prevalence of CCP are not well elucidated. Nevertheless, it is known that tuberculosis is the most frequent etiology of constrictive pericarditis in Africa and emerging countries (40–90%) [16] versus other rare etiology in our practice such as constrictive pericarditis after surgery. In a recent study, Gaudino et al. [17] have concluded their study insisting on the fact that constrictive pericarditis after surgery has its own pathophysiological characteristics, but we still be ignorant on its real origin and pathogenesis. Therefore, we should be vigilant and keep in mind that any cardiac symptoms without explanation must be suspected and be treated surgically as soon as possible if there is any doubt of pericardial constriction.

### **6. Diagnosis**

In African countries [18–26], the diagnosis of constrictive pericarditis is usually obvious for patients present late after the development of the constrictive process characterized mostly by advanced clinical manifestations of right-sided heart failure (50–100%), progressive New York Heart Association (NYHA) Functional Class III or IV (42–100%) associated with an evident antecedent pulmonary and extra-pulmonary tuberculosis such as tuberculous pericarditis (26–99%). Duration of illness prior to surgery may range from 1 month to 25 years with an average of 15 or 30 months found, respectively, by Ali et al. [26] and Yangni-Angate et al. [21].

Generally, male gender predominance is noted (60–80%), patients have an average age below 40 years; for instance in Morocco (32 years) [18], Ghana (33 years) [22], Senegal (23 years) [20], Gabon (36 years) [23], Cote d'Ivoire (28.8 years) [21], and Ethiopia (24.3 years) [26]. Main clinical findings often due to severe constriction include: hepatomegaly (74–100%), raised jugular venous pressure (76–100%), hepato-jugular reflux (67–100%), ascite (50–76%), peripheral edema (46–79%), complete "Pick Syndrome"(50–76%). Kussmaul sign is less detected (9.8%). In African setting, frequent radiographical findings at chest X-ray are as follows: enlarged cardiothoracic ratio or cardiomegaly (88–47%), calcifications (36–52.5%), and pleural effusion (44–63%); electrocardiography shows invariable modifications such as low QRS voltage (32.5–82.5%), atrial fibrillation (18–46%), and T wave abnormality up to 87.5%. Echocardiography is largely available and is useful for accurate assessment in revealing in most cases a thickened pericardium (56 and 100%), pericardial calcifications in 30.2%, 47.4% of cases according to authors and left ventricular septal motion abnormality (63.6%) with reduced ejection fraction below 0.60 in Rabat, Morocco [19]; cardiac catheterization performed only in a very few centers [21, 24, 25, 27] documents elevation and equalization of diastolic heart pressures with the typical dip-plateau waveform of constrictive pericarditis and evaluate the stroke volume, cardiac output, and myocardial systolic function. It still prevails to be the most final diagnostic assessment in sub-Saharan Africa. In his study, Yangni-Angate et al. [21] reported that cardiac catheterization confirmed a dip-and-plateau (square root sign), an equalization of end-diastolic pressures in right and/or left cardiac chambers ranged between 10 and 40 mmHg, a mean cardiac index (CI): 2.3 l/min/m2 (extremes: 1.3–3.6). From this author, the constriction was limited to the right cardiac cavities called right constriction (n = 54, 45%) or to the right and left cardiac cavities called bilateral constriction (n = 66, 55%) and hemodynamic parameters and cineangiograms confirmed the diagnosis of pericardial constriction in all the patients. Omboga in Nairobi [25] showed the same observation with elevation of intracardial pressures in all cases, raised mean right atrium, enddiastolic right and left ventricular, and elevated mean pulmonary artery pressures at 18, 18, 20, and 27mmHg, respectively. Other imaging studies such as computed tomography and magnetic resonance imaging are rarely prescribed because they are inexistent usually. Those modern imaging techniques could be heavily useful in diagnosing constrictive pericarditis. When done, pericardial biopsy can be contributive for constrictive pericarditis etiology. Laboratory investigations regarding protein-losing enteropathy in patients with chronic constrictive pericarditis are not yet done for Africans. Differential diagnosis is always considered, and distinction from both constrictive pericarditis and restrictive cardiomyopathy due to endomyocardial fibrosis is really the usual situation to be clarified. Endomyocardial fibrosis (EMF) is a tropical heart disease with fibrous endocardial lesions lying in the right and/or the left ventricle. Endoventricular fibrosis is shown and confirmed in all cases by bi-dimensional echocardiography and angiocardiography [28].

### **7. Natural history**

Constrictive pericarditis occurs mostly in our African context after a nondiagnosed, untreated tuberculous or pyogenic pericarditis or even as a sequel of a treated tuberculous pericarditis. Constrictive process starts with an acute pericarditis; then a subacute and chronic pericarditis marked in most of the cases by a fusion of the two layers of the pericardium and an occlusion of the pericardial cavity [3].

The delay from onset clinical symptoms to constriction is widely flexible from 1 month to 10 years even 25 years [2].

**45**

Senegal by Ciss [20].

*Chronic Constrictive Pericarditis (CCP) in Africa: Epidemiology, Etiology, Diagnosis…*

When diagnosis of constrictive pericarditis is confirmed and surgery is indicated, a pericardiectomy should not be delayed; surgery remains the only efficient and comprehensive treatment option. Pericardiectomy is frequently performed via a median sternotomy approach or a left anterolateral thoracotomy approach; it may be partial or complete. From the African teams' surgical experiences, cardiopulmonary bypass has not been used in all cases and excellent early surgical outcomes were reported.

Yangni-Angate in Cote d'Ivoire [21], in his retrospective study related to 120 patients with CCP who underwent pericardiectomy through a median sternotomy approach (n = 117; 97.5%) found 15 early deaths (12.5%); the cause of hospital deaths was due to a low cardiac output (n = 12) and to a hepatic failure (n = 3). Class III or IV (NYHA) (p = 0.01), mitral regurgitation (p < 0.05), persistent a diastolic syndrome after surgery (p < 0.05) and low cardiac index (p < 0.02) were the important risk factors (**Table 1**). Age, size of cardiac X-ray silhouette, right and left ventricular diastolic pressures, ejection fraction, atrial fibrillation, and pericardial

Tettey in Ghana [22] reviewed the surgical management of constructive pericarditis and the post-operative challenges of 11 patients who had pericardiectomy via a median sternotomy in all patients with no early mortality and a significant improve-

Mutyaba in South Africa [24] through a retrospective study of 121 patients who had undergone total (n = 105; 88.2%) or partial (n = 14; 11.8%) pericardiectomy for constrictive pericarditis at Groote Schuur Hospital, noted an early mortality of 14% (n = 14) mainly due to a low cardiac output syndrome. In this work, it has been statistically attested that serum sodium and pre-operative New York Heart Association Class IV versus combined Class I–III were independent predictors of early mortality. He also showed that early mortality after pericardiectomy was not influenced by HIV status and that of New York Heart Association Functional Class IV and hyponatremia were predictable factor for early mortality after pericardiectomy.

Ali in Ethiopia [26] has done a retrospective study at the Thoracic Surgical Unit, Tikur Anbessa Hospital, Department of Surgery, Medical Faculty, Addis Ababa University, Addis Ababa on 19 patients who underwent pericardiectomy for CCP by a median sternotomy approach (n = 15; 79%) often. One early post-operative mortality was registered. The author emphasized the benefit of pericardiectomy in

Ondo N'Dong in Gabon [23] has published his series on 18 patients with constrictive pericarditis treated surgically. All of them underwent a partial pericardiectomy via a left anterior thoracic incision in 17 patients and a median sternotomy incision in 1 patient. Four patients died in the early post-operative period due to low cardiac output; this study revealed pre-operative severe heart failure as a principal predictable risk factor for early death after pericardiectomy. This finding has been also noted In Rabat, Morocco by Nzondo [19]; in Fes, Morocco by Hind [18] and in

Nzondo [19] has retrospectively analyzed 11 patients who had undergone partial pericardiectomy via a median sternotomy approach for constrictive pericarditis. Early mortality was of 9.1% related to acute heart and multi-organs failure. Hind in

*DOI: http://dx.doi.org/10.5772/intechopen.84887*

**9.1 From cardiac centers in Africa**

**9. Early outcomes and risk factors for early deaths**

calcifications had no impact on early survival (**Table 2**).

terms of physical exercise improvement.

ment of functional capacity of all of the patients followed-up.

**8. Surgical treatment**

*Chronic Constrictive Pericarditis (CCP) in Africa: Epidemiology, Etiology, Diagnosis… DOI: http://dx.doi.org/10.5772/intechopen.84887*
