Clinical Case of Rare Acute and Chronic Cardio-Pulmonary Involvement in Neurofibromatosis Type-1 (Von Recklinghausen’s Disease)
Nicola Vitulano1*, Giuseppe Galgano1, Enrico Maria Pellegrini2, Vito Casamassima1, Pietro Schino3, Massimo Grimaldi1, Tommaso Langialonga1
1Department of Cardiology, Ente Ecclesiastico Ospedale Regionale “F. Miulli”, Acquavivadelle Fonti (Ba), Italy
2Department of Cardiology, Louis Pasteur University Hospital, Kosice, Slovak Republic
3 Respiratory Physiopathology Unit, Ente Ecclesiastico Ospedale Regionale “F. Miulli”, Acquavivadelle Fonti (Ba), Italy
*Corresponding author: Nicola Vitulano, Department of Cardiology, Ente Ecclesiastico Ospedale Regionale “F. Miulli”, Acquavivadelle Fonti (Ba),Italy. Tel: +393475600196; Email: nicola.vitulano@gmail.com
Received Date: 26 January, 2018; Accepted Date: 29 January, 2018; Published
Date: 07 February, 2018
Citation: Vitulano N, Galgano G, Pellegrini EM, Casamassima V, Schino P., Grimaldi M, et al. (2018) Clinical Case of Rare Acute and Chronic Cardio-Pulmonary Involvement in Neurofibromatosis Type-1 (Von Recklinghausen’s Disease). Ann Case Rep: ACRT-158. DOI: 10.29011/2574-7754/100058
1.
Introduction
A
58-year-old woman with von Recklinghausen’s disease (Neurofibromatosis Type 1)
was referred to our Intensive Care Unit for progressive dyspnea at rest,
diaphoresis and hypotension. Objective examination showed reduced intensity of
cardiac tones, with diffuse reduction of pulmonary murmurs without signs of pulmonary
edema. ECG findings revealed sinus tachycardia, 115 bpm, with low voltage of
QRS (Figure 1).
3.
Discussion
Figure
1:
ECG at the moment of hospitalization.
Figure 2: Echocardiogram: severe pericardial effusion (“Swimming Heart”).
|
|
Basal |
3 Month Control |
12 Month Control |
WHO-FC |
|
III-IV |
III |
II-III |
Echocardiography |
TVR (m/sec) |
4,3 |
2,9 |
3,2 |
TAPSE (mm) |
12 |
16 |
16 |
|
Pericardial Effusion (Post Pericardiocenteis) |
Mild |
Mild |
None |
|
Chateterization |
MPAP (mmHg) |
59 |
44 |
32 |
RAP (mmHg) |
13 |
4 |
1 |
|
PAWP (mmHg) |
12 |
15 |
4 |
|
CO (l/min) |
5.33 |
8.6 |
8,1 |
|
CI (l/min/m²) |
3.15 |
4.91 |
4,96 |
|
PVR |
8.81 WU 704.82 dyne×sec/cm5 |
3.3 WU 264 dyne×sec/cm5 |
3.4 WU 272dyne×sec/cm5 |
|
PVRI |
14.91 WU×m2; 1.192.9dyne×sec*m²/cm5 |
5.8 WU×m²; 464dyne×sec*m²/cm5 |
5.6 WU×m²; 448dyne×sec*m²/cm5 |
|
WHO-FC:World Health Organization Functional Class; TVR:Peak Tricuspid Regurgitation Velocity; TAPSE: Tricuspid Annular Plane Systolic Excursion; MPAP: Mean Pulmonary Artery Pressure; RAP: Right Atrial Pressure; PAWP: Pulmonary Capillary Wedge Pressure; CO: Cardiac Output; CI: Cardiac Index; PVR: Pulmonary Vascular Resistance; PVRI: Pulmonary Vascular Resistance Index |
Table 1: Stabilized clinical setting invasive hemodynamic evaluation was consistent with pre-capillary pulmonary hypertension with preserved cardiac output (mPAP 59 mmHg, PVR 8.81 WU).
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