1. WORKSHOP 12 III-C6 Matematico  Matias  Maulion  Medenilla  Medina, K.  Medina, S.  Mejino  Melgarejo  Mendoza, A

2. MM 23 years old Female

3. 4 Years PTA Irregular palpitations heart beats associated with increased sweating and SOB Patient consulted and given Verapamil for which she took for only three days and would take it intermittently for palpitation Few Hours PTA Palpitation accompanied by SOBCONSULT AND ADMISSION

4. Conscious, coherent, ambulatory BP: 110/70 CR 80/min regular RR 16/min BMI 19 Symmetrical chest expansion, narrow A-P diameter of the chest, no retractions, clear breath sounds Adynamic precordium, AB at 5 th LICS MCL, no murmurs, (+) midsystolic click followed by 2/6 midsystolic cresecendo murmur noted at the apex

5.  (-) dizziness  (-) loss of consciousness  (-) cough or colds  (-) PND or Orthopnea

6.  2D Echo-Doppler: MVP, anterior mitral valve leaflet with moderate MR, slightly dilated LA with no evidence of thrombus  123L ECG: sinus rhythm, occasional premature atrial complexes  CXR: Normal

8.  Body weight is often low ; asthenic  Blood pressure is usually normal or low  Orthostatic hypotension  Resting bradycardia  Thoracic skeletal abnormalities suggesting MVP:  Scoliosis, pectus excavatum, straightened thoracic spine, and narrowed AP dm of the chest Zippes, D., et al. (2005) Braunwald’s Heart Disease: A textbook of Cardiovascular Medicine, 7 th ed. USA: Elsevier Saunders

9.  Auscultation: ▪ Nonejection systolic click at least 0.14 sec after S1 ▪ Multiple mid and late systolic clicks along the L lower sternal border ▪ Mid to late crescendo systolic murmur that continues to A2 Zippes, D., et al. (2005) Braunwald’s Heart Disease: A textbook of Cardiovascular Medicine, 7 th ed. USA: Elsevier Saunders

10.  Dynamic Auscultation: End-diastolic LV volume Critical LV volume is achieved earlier in systole Click-murmur moves closer to S1 LV systolic volume/ afterload Lengthens the time from onset of the systole to MVP Click-murmur moves closer to S2 Zippes, D., et al. (2005) Braunwald’s Heart Disease: A textbook of Cardiovascular Medicine, 7 th ed. USA: Elsevier Saunders

11.  Dynamic Auscultation: Response of the Murmur of MVP to Interventions InterventionTimingIntensity Standing upright Recumbent or 0 Squatting or 0 Hand grip + Valsalva + Amyl nitrite + Fuster, V.,et al. (2008). Hurst’s The Heart, 12the ed. China: McGrawHill Co.

12.  Physical  Thin aesthetic body habitus with narrow anteroposterior diameter 12  Skeletal abnormalities (ie, pectus excavatum, straight back, kyphoscoliosis)  Supernumerary nipples in Asian Indians  Resting bradycardia and orthostatic hypotension  Cardiac auscultation  Apical, single or multiple, mid-to-late systolic clicks, which result from the tightening of the chordae tendineae or the redundant valve, can be heard.  An apical mid-to-late systolic murmur of crescendo, decrescendo, or constant nature can be heard, and the murmur continues to be heard in S 2.  The click and the murmur change as the position changes (closer to S 1 with diminished LV volume; closer to S 2 with increased LV volume)  In the supine position, the click is late (ie, close to S 2 ), and the murmur is brief.  In the standing position and during the Valsalva maneuver, the click is earlier (ie, close to S 1 ), and the murmur is longer. This may identify a murmur that previously was not noted.

13.  In the squatting position, the click is later (ie, closer to S 2 ), and the murmur is shorter. The click and the murmur may even disappear.  The isometric handgrip exercise increases the intensity (ie, loudness) of the murmur without affecting the position.  The murmur should be distinguished from that of aortic stenosis (ie, early systolic, at base); pulmonic flow murmur (ie, short and early systolic, diminishes with Valsalva maneuver); hypertrophic cardiomyopathy (ie, diminishes with squatting and intensifies with standing and Valsalva maneuver); and mitral regurgitation (ie, holosystolic murmur with S 3, enlarged and displaced point of maximal intensity [PMI]). .

14.  Mitral regurgitation  Autonomic dysfunction - Decreased heart rate variability and parasympathetic tone 13,14  Neuroendocrine dysfunction  Ehlers-Danlos syndrome findings (eg, joint hypermobility, abnormal striae, bruising, distensibility of skin)  Osteogenesis imperfecta findings (eg, blue sclera)  Marfan syndrome findings (eg, scoliosis, straight back, pectus excavatum, arachnodactyly, arm span greater than body height)  Stickler syndrome findings (eg, kyphosis, scoliosis, mandibular hypoplasia, retinal detachment). Whether Stickler syndrome is associated with MVP is debatable

16. COMMON  Easy fatigability  Shortness of breath  Palpitation  Chest pain  Light-headedness  Syncope UNCOMMON  TIA  Congestive Heart Failure  Endocarditis  in MR associated with MVP  Sudden death

17. associated with AUTONOMIC DYSFUNCTION are associated with GENETICALLY INHERITED MVP:  Anxiety  Panic attacks  Exercise intolerance  Palpitations (may be a symptom of benign arrythmias)  Atypical chest pain  Fatigue  Orthostasis  Syncope or presyncope  Neuropsychiatric symptoms

18.  Fatigue  Dyspnea  Exercise intolerance  Orthopnea  Paroxysmal nocturnal dyspnea Related to progression of Mitral Regurgitation:

20.  Severe Mitral Regurgitation  over years or decades, due to chordal rupture and massive prolapse of both leaflets  rapidly, due to chordal or endocarditis  Arrythmias  most commonly ventricular premature contractions and paroxysmal supraventricular and ventricular tachycardia

21.  Transient cerebral ischemic attacks  secondary to emboli from the mitral valve due to endothelial disruption have been reported,  Infective endocarditis  may occur in patients with MR and/or leaflet thickening

23.  Greater frequency among those with Collagen (type 3) Disorders:  Marfan’s Syndrome  Osteogenesis Imperfecta  Ehlers-Danlos Syndrome