[CASE] SOB in a patient with history of lobectomy in childhood
Man in his mid-forties presented to GP office with lower limbs edema and ascites which had developed within a month. He admitted that he had been experiencing exertional dyspnoea for about a year. PMH included well-controlled hypertension and left lower lobectomy with removal of VI-VIII ribs because of a tumor in childhood - there was no documentation available. He was reffered immediately to the hospital.
This ECG was taken in ED.
While no voltage and morphologic criteria for right ventricle hypertrophy (RVH) were met, presence of right atrial enlargement and RV strain pattern may support such diagnosis.
Lab tests showed highly elevated NT-proBNP level and normal renal function.
Echo examination showed this image.
CT angiography was done to exclude PE. There was no sign of pulmonary embolism but stenosis of 6th segment bronchus with accompanying emphysema was present.
Initial diagnosis of right ventricular failure due to pulmonary hypertension (PH) was made. Patient was admitted to cardiology ward and put on intravenous loop diuretic and oral mineralocorticoid receptor antagonist.
Patient was stable and there was satisfying weight loss with reduction of peripheral edema within the next few days and referral to specialist pulmonary hypertension center for right heart catheterization (RHC) was planned.
Unfortunately during the morning visit patient had a cardiac arrest (PEA rhythm) and was transferred to ICU in cardiogenic shock after return of spontaneous circulation. Lab tests run before SCA indicated acute renal failure. Later on patient developed septic shock and did not make a recovery...
A few studies showed that pneumonectomy or major lung resection may be associated with increased pulmonary artery pressure and development of pulmonary hypertension.
Increase in pressure load causes RV adaptation by increasing contractility and wall thickening (hypertrophy) and later on dilating to maintain stroke volume which ultimately leads to its failure. RV function in PH has great impact on mortality thus making diagnosis of pulmonary hypertension early and introducing treatment to reduce RV afterload (if possible) is vital. Some treatment strategies of RV failure (e.g. atrial septostomy or mechanical-assist device) still need to be further investigated so they can be applied in clinical practice.
Further reading:
1. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT), European Heart Journal, Volume 37, Issue 1, 1 January 2016, Pages 67–119, https://doi.org/10.1093/eurheartj/ehv317
2. Foroulis et al. Study on the late effect of pneumonectomy on right heart pressures using Doppler echocardiography, European Journal of Cardio-Thoracic Surgery, Volume 26, Issue 3, September 2004, Pages 508–514, https://doi.org/10.1016/j.ejcts.2004.05.036
3. Potaris et al. (2014). Pulmonary hypertension after pneumonectomy for lung cancer. Asian Cardiovascular and Thoracic Annals, 22(9), 1072–1079. https://doi.org/10.1177/0218492314527992
4. Hill, Nicholas & R Roberts, Kari & Preston, Ioana. (2009). Postoperative Pulmonary Hypertension: Etiology and Treatment of a Dangerous Complication. Respiratory care. 54. 958-68. 10.4187/002013209793800439.
This ECG was taken in ED.
Strips show sinus tachycardia 100 bpm and normal axis. There is possible right atrial enlargement (P around 2,5 mm in II and 1,5mm in V1) and downward ST depression with biphasic T wave in inferior leads (II, III, aVF) - right ventricular strain pattern.
While no voltage and morphologic criteria for right ventricle hypertrophy (RVH) were met, presence of right atrial enlargement and RV strain pattern may support such diagnosis.
Lab tests showed highly elevated NT-proBNP level and normal renal function.
Echo examination showed this image.
You can see dilatation and hypertrophy of hypokinetic right ventricle (on the right side) and dilatation of right atrium. Left ventricle seems to be normal size and ejection fraction is preserved.
Using tricuspid regurgitant jet velocity pulmonary artery systolic pressure (PASP) was estimated to be around 70 mmHg which alone suggested high probability of pulmonary hypertension. No left heart valves disease nor shunt was found.
CT angiography was done to exclude PE. There was no sign of pulmonary embolism but stenosis of 6th segment bronchus with accompanying emphysema was present.
There is severe right scoliosis with spinal stabilization and deformation of rib cage.
Huge right atrium (RA) with leftward bulging of interatrial septum (IAS) - sign of significantly increased right atrial pressure.
Gigantic main pulmonary artery (MPA) and right pulmonary artery (RPA). Left pulmonary artery diameter was within normal range.
Initial diagnosis of right ventricular failure due to pulmonary hypertension (PH) was made. Patient was admitted to cardiology ward and put on intravenous loop diuretic and oral mineralocorticoid receptor antagonist.
Patient was stable and there was satisfying weight loss with reduction of peripheral edema within the next few days and referral to specialist pulmonary hypertension center for right heart catheterization (RHC) was planned.
Unfortunately during the morning visit patient had a cardiac arrest (PEA rhythm) and was transferred to ICU in cardiogenic shock after return of spontaneous circulation. Lab tests run before SCA indicated acute renal failure. Later on patient developed septic shock and did not make a recovery...
A few studies showed that pneumonectomy or major lung resection may be associated with increased pulmonary artery pressure and development of pulmonary hypertension.
Increase in pressure load causes RV adaptation by increasing contractility and wall thickening (hypertrophy) and later on dilating to maintain stroke volume which ultimately leads to its failure. RV function in PH has great impact on mortality thus making diagnosis of pulmonary hypertension early and introducing treatment to reduce RV afterload (if possible) is vital. Some treatment strategies of RV failure (e.g. atrial septostomy or mechanical-assist device) still need to be further investigated so they can be applied in clinical practice.
Further reading:
1. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT), European Heart Journal, Volume 37, Issue 1, 1 January 2016, Pages 67–119, https://doi.org/10.1093/eurheartj/ehv317
2. Foroulis et al. Study on the late effect of pneumonectomy on right heart pressures using Doppler echocardiography, European Journal of Cardio-Thoracic Surgery, Volume 26, Issue 3, September 2004, Pages 508–514, https://doi.org/10.1016/j.ejcts.2004.05.036
3. Potaris et al. (2014). Pulmonary hypertension after pneumonectomy for lung cancer. Asian Cardiovascular and Thoracic Annals, 22(9), 1072–1079. https://doi.org/10.1177/0218492314527992
4. Hill, Nicholas & R Roberts, Kari & Preston, Ioana. (2009). Postoperative Pulmonary Hypertension: Etiology and Treatment of a Dangerous Complication. Respiratory care. 54. 958-68. 10.4187/002013209793800439.
5. Berend E. Westerhof, Nabil Saouti, Willem J. van der Laarse, Nico Westerhof, Anton Vonk Noordegraaf, Treatment strategies for the right heart in pulmonary hypertension, Cardiovascular Research, Volume 113, Issue 12, October 2017, Pages 1465–1473, https://doi.org/10.1093/cvr/cvx148
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