Pulmonary Hypertension in Thalassemia Patients

Pulmonary hypertension (PH) is defined in children as a mean pulmonary arterial pressure (PAP) greater than 25 mmHg at rest or 30 mmHg during physical activity, with increased pulmonary artery capillary wedge pressure and an increased pulmonary vascular resistance greater than 3 Wood units × M2. it is the main cause of morbidity and mortality in the group of thalassemia, if no treatment leads to right ventricular heart failure and death. The development of pulmonary arterial hypertension (PAH) is assumed to be the result of many multifactorial pathogenic mechanisms including chronic hemolysis, iron overload, hypercoagulability, and erythrocyte dysfunction as a result of splenectomy, inflammation and nitric oxide (NO) depletion. PAH symptoms are non-specific, their signs consist of right ventricular lift, an accentuated pulmonary component of the second heart sound, a (gallop rhythm) right ventricular third heart sound, and parasternal heave meaning a hypertrophied right ventricle. The diagnosis of PAH requires a clinical suspicion based on symptoms and physical examination. Echocardiography is frequently used to screen for PAH, monitor progression over time and allow identification of patients for whom diagnostic right heart catheterization (RHC) is warranted and its treatment includes hemoglobinopathy specific treatment and PAH specific therapy.

Additional Heart Sounds—Part 1 (Third and Fourth Heart Sounds)

S3 is a low-pitched sound (25–50Hz) which is heard in early diastole, following the second heart sound. The following synonyms are used for it: ventricular gallop, early diastolic gallop, protodiastolic gallop, and ventricular early filling sound. The term “gallop” was first used in 1847 by Jean Baptiste Bouillaud to describe the cadence of the three heart sounds occurring in rapid succession. The best description of a third heart sound was provided by Pierre Carl Potain who described an added sound which, in addition to the two normal sounds, is heard like a bruit completing the triple rhythm of the heart (bruit de gallop). The following synonyms are used for the fourth heart sound (S4): atrial gallop and presystolic gallop. S4 is a low-pitched sound (20–30 Hz) heard in presystole, i.e., shortly before the first heart sound. This produces a rhythm classically compared with the cadence of the word “Tennessee.” One can also use the phrase “A-stiff-wall” to help with the cadence (a S4, stiff S1, wall S2) of the S4 sound.

Second Heart Sound

The auscultation of second heart sound reveals presence of the disease and its pathophysiology, conduction defects, ventricular function, and hemodynamics around the aortic and pulmonary valves. To understand the splitting of the second heart sound, it is useful to understand the concepts of hangout interval and ejection time. Differential diagnosis of S2 split include opening snap, third heart sound, and pericardial knock. Variations of second heart sound may involve intensity or variations of split. Variations of split can be single heart sound, wide split, wide fixed split, or paradoxical splitting.Interpretation of second heart sound in congenital heart disease provides information about the presence and position of semilunar valves.

Acute cardiac failure: Definitions, investigation, and management

Presentations of acute heart failure fall into three overlapping categories: acute breathlessness and pulmonary oedema; chronic fluid retention and peripheral oedema (anasarca); and cardiogenic shock. Features on examination include tachycardia, hypotension, a raised venous pressure, basal crackles, and peripheral oedema. Auscultation may reveal a third heart sound or features of valvular heart disease. Initial management focuses on confirming the diagnosis and identification of the immediate precipitant (e.g. arrhythmias, myocardial infarction, decompensating valvular heart disease). Initial investigations include a 12-lead electrocardiogram, chest radiograph, full blood count, biochemical screen, troponin, and thyroid function. Natriuretic peptides are useful in confirming the diagnosis where clinical features are present and a normal level of these is helpful in excluding the diagnosis. All patients should undergo echocardiographic assessment early in the course of a hospital admission to assess left ventricular function and to look for underlying valvular heart disease.

Chronic heart failure diagnosis: symptoms, signs, and the ECG

The clinical history aims to elicit the symptoms that are commonly experienced by patients with heart failure such as dyspnoea, paroxysmal nocturnal dyspnoea, orthopnoea, and fatigue. The history may also provide clues as to the aetiology of heart failure and symptoms that suggest alternative diagnoses. Similarly, signs that are elicited on clinical examination are used to support the diagnosis of heart failure. In addition, they can be used to determine prognosis and assess response to treatment or the need for more intensive treatment. Common signs such as peripheral oedema, jugular venous distension, and pulmonary crackles are less specific for the diagnosis of heart failure than others such as a third heart sound. The presence of some clinical signs may help determine the aetiology of heart failure and indicate other potential diagnoses that may present like heart failure but require very different treatment. While signs and symptoms are used in conjunction with imaging evidence and raised natriuretic peptides to make the diagnosis of heart failure, the electrocardiogram (ECG) still plays a central role. The ECG can be used to determine underlying aetiology (such as evidence of prior myocardial infarction) and guide therapeutic decision-making such as the need for cardiac resynchronization therapy in those with bundle branch block. Information on symptoms, signs, and investigations such as the ECG need to be integrated to ensure the accurate diagnosis and optimal treatment of patients with heart failure.