History

Fact Explanation
Difficulty in breathing [1] Hypoxemia due to hypoventilation in kyphoscoliosis leads to pulmonary vasoconstriction and pulmonary hypertension which give rise to difficulty in breathing [1]
Fatigue [1] Reduced oxygen to tissues due to hypoxaemia [1]
Ankle swelling [2] Kyphoscoliotic diseases leads to pulmonary hypertension,which increases the work of the right ventricle, which leads more or less rapidly to right ventricular enlargement (associating hypertrophy and dilatation) which can result in ventricular dysfunction (systolic, diastolic). Later, right heart failure (RHF) ) characterised by the presence of peripheral edema [2]
History of chronic obstructive pulmonary disease / interstitial lung disease [3] These can lead to pulmonary hypertension due to changes in lung tissues [3]
History of Ehlers-Danlos Syndrome [4] Ehlers-Danlos syndrome (EDS), kyphoscoliotic form (previously known as EDS VI) is a generalized connective tissue disorder.and respiratory compromise if kyphoscoliosis is severe [4]
Family history [4] Kyphoscoliosis can be seen in several inherited connective tissue disorders.eg: EDS, kyphoscoliotic form is inherited in an autosomal recessive manner [4]
References
  1. HECHT H. H.. Heart Failure and Lung Disease. Circulation [online] 1956 August, 14(2):265-290 [viewed 09 July 2014] Available from: doi:10.1161/​01.CIR.14.2.265
  2. WEITZENBLUM E.. CHRONIC COR PULMONALE. [online] 2003 February, 89(2):225-230 [viewed 09 July 2014] Available from: doi:10.1136/heart.89.2.225
  3. SHUJAAT A, MINKIN R, EDEN E. Pulmonary hypertension and chronic cor pulmonale in COPD Int J Chron Obstruct Pulmon Dis [online] 2007 Sep, 2(3):273-282 [viewed 09 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2695205
  4. Yeowell HN, Steinmann B. Ehlers-Danlos Syndrome, Kyphoscoliotic Form. 2000 Feb 2 [viewed 09 July 2014] Available from: http://www.ncbi.nlm.nih.gov/books/NBK1462/

Examination

Fact Explanation
Dyspnoea [1] Hypoxemia due to hypoventilation in kyphoscoliosis leads to pulmonary vasoconstriction and pulmonary hypertension which give rise to difficulty in breathing [1]
Spinal defects [1] Connective tissues disorders [1] Kyphosis is excessive curvature of the spine in the sagittal (A-P) plane. Scoliosis is abnormal curvature of the spine in the coronal (lateral) plane. Lordosis or hyperlordosis is excessive curving of the lower spine and is often associated with scoliosis or kyphosis Kyphoscoliosis A scoliosis accompanied by a true hyperkyphosis [2]
Barrel shaped chest [3] Due to increased anteroposterior diameter [3]
Friable, hyperextensible skin, thin scars, easy bruising [4] Skin changes in Ehlers-Danlos Syndrome [4]
Ankle oedema , ascites [5] Kyphoscoliotic diseases leads to pulmonary hypertension,which increases the work of the right ventricle, which leads more or less rapidly to right ventricular enlargement (associating hypertrophy and dilatation) which can result in ventricular dysfunction (systolic, diastolic). Later, right heart failure (RHF) ) characterised by the presence of peripheral edema [5]
Engorged neck veins [5] Due to right heart failure [5]
A murmur of tricuspid regurgitation [1] Pansystolic heart murmur.Best heard over lower left sternal border.Suggesting right ventricular dilatation, is a very late sign in respiratory patients [1]
Changes in heart sounds [1] Due to pulmonary hypertension.Widely split S2 or second heart sound, a loud P2 or pulmonic valve closure sound [1]
Cyanosis [1] Hypoxemia due to hypoventilation [1]
Polycythemia [1] Arterial oxygen desaturation causes a relative and absolute increase in red cell mass: erythrocytosis (secondary polycythemia) [1]
References
  1. HECHT H. H.. Heart Failure and Lung Disease. Circulation [online] 1956 August, 14(2):265-290 [viewed 09 July 2014] Available from: doi:10.1161/​01.CIR.14.2.265
  2. SMITH JUSTIN S., SHAFFREY CHRISTOPHER I., FU KAI-MING G., SCHEER JUSTIN K., BESS SHAY, LAFAGE VIRGINIE, SCHWAB FRANK, AMES CHRISTOPHER P.. Clinical and Radiographic Evaluation of the Adult Spinal Deformity Patient. Neurosurgery Clinics of North America [online] 2013 April, 24(2):143-156 [viewed 10 July 2014] Available from: http://rockymountainorthopedics.com/util/documents/scoliosis-spine/Clinical_and_Radiographic_NeurosurgClinNam_april2013.pdf
  3. PUNTURIERI A, CROXTON TL, WEINMANN G, KILEY JP. The changing face of COPD. Am Fam Physician [online] 2007 Feb 1, 75(3):315-6 [viewed 09 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/17304861
  4. Yeowell HN, Steinmann B. Ehlers-Danlos Syndrome, Kyphoscoliotic Form. 2000 Feb 2 [viewed 09 July 2014] Available from: http://www.ncbi.nlm.nih.gov/books/NBK1462/
  5. WEITZENBLUM E.. CHRONIC COR PULMONALE. [online] 2003 February, 89(2):225-230 [viewed 09 July 2014] Available from: doi:10.1136/heart.89.2.225

Differential Diagnoses

Fact Explanation
Right-sided heart failure due to right ventricular myocardial infarction [1] Will present with severe chest pain and Electrocardiogram will show changes in ST segments [1]
Dilated Cardiomyopathies [2] Can cause heart failure.Characterized by ventricular dilation and depressed myocardial contractility in the absence of abnormal loading conditions such as hypertension or valvular disease [2]
Tachycardia-Induced Cardiomyopathy [2] Reversible cause of heart failure.Patient will have arrhythmia [2]
Myocarditis [2] Inflammation of the heart may cause heart failure.Presentation may be acute, with a distinct onset, severe hemodynamic compromise, and severe left ventricular dysfunction [2]
Congenital heart defects [2] Can cause heart failure.These defects can be identified in an echocardiogram [2]
Hypertension induced heart failure [2] Patient will have a history of hypertension as well as high blood pressure levels on examination [2]
Valvular defects of heart [2] Can cause heart failure.These can be identified in an echocardiogram [2]
References
  1. VOELKEL N. F., QUAIFE R. A., LEINWAND L. A., BARST R. J., MCGOON M. D., MELDRUM D. R., DUPUIS J., LONG C. S., RUBIN L. J., SMART F. W., SUZUKI Y. J., GLADWIN M., DENHOLM E. M., GAIL D. B.. Right Ventricular Function and Failure: Report of a National Heart, Lung, and Blood Institute Working Group on Cellular and Molecular Mechanisms of Right Heart Failure. Circulation [online] 2006 October, 114(17):1883-1891 [viewed 10 July 2014] Available from: doi:10.1161/​CIRCULATIONAHA.106.632208
  2. YANCY C. W., JESSUP M., BOZKURT B., BUTLER J., CASEY D. E., DRAZNER M. H., FONAROW G. C., GERACI S. A., HORWICH T., JANUZZI J. L., JOHNSON M. R., KASPER E. K., LEVY W. C., MASOUDI F. A., MCBRIDE P. E., MCMURRAY J. J. V., MITCHELL J. E., PETERSON P. N., RIEGEL B., SAM F., STEVENSON L. W., TANG W. H. W., TSAI E. J., WILKOFF B. L.. 2013 ACCF/AHA Guideline for the Management of Heart Failure: A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation [online] December, 128(16):e240-e327 [viewed 10 July 2014] Available from: doi:10.1161/​CIR.0b013e31829e8776

Investigations - for Diagnosis

Fact Explanation
Electrocardiogram [1] Right ventricular hypertrophy- Right axis deviation (> 90 degrees) in presence of disease capable of causing RVH R in aVR ≥ 5 mm, or R in aVR > Q in aVR Any one of the following in lead V1: R/S ratio > 1 and negative T wave qR pattern R > 6 mm, or S < 2mm, or rSR' with R' > 10 mm [1]
Chest x ray [1] Right atrial and ventricular enlargement [1]
Echocardiogram [2] Wall thickness,chamber dilatations,ejection fraction can be measured.Valve insufficiencies can be identified [2]
References
  1. HECHT H. H.. Heart Failure and Lung Disease. Circulation [online] 1956 August, 14(2):265-290 [viewed 09 July 2014] Available from: doi:10.1161/​01.CIR.14.2.265
  2. VOELKEL N. F., QUAIFE R. A., LEINWAND L. A., BARST R. J., MCGOON M. D., MELDRUM D. R., DUPUIS J., LONG C. S., RUBIN L. J., SMART F. W., SUZUKI Y. J., GLADWIN M., DENHOLM E. M., GAIL D. B.. Right Ventricular Function and Failure: Report of a National Heart, Lung, and Blood Institute Working Group on Cellular and Molecular Mechanisms of Right Heart Failure. Circulation [online] 2006 October, 114(17):1883-1891 [viewed 10 July 2014] Available from: doi:10.1161/​CIRCULATIONAHA.106.632208

Investigations - Fitness for Management

Fact Explanation
Full blood count [1] To assess general condition of the patient and hemoglobin levels. [1]
References
  1. HECHT H. H.. Heart Failure and Lung Disease. Circulation [online] 1956 August, 14(2):265-290 [viewed 09 July 2014] Available from: doi:10.1161/​01.CIR.14.2.265

Investigations - Followup

Fact Explanation
Electrocardiogram [1] Right ventricular hypertrophy- Right axis deviation (> 90 degrees) in presence of disease capable of causing RVH R in aVR ≥ 5 mm, or R in aVR > Q in aVR Any one of the following in lead V1: R/S ratio > 1 and negative T wave qR pattern R > 6 mm, or S < 2mm, or rSR' with R' > 10 mm [1]
Echocardiogram [2] Wall thickness,chamber dilatations,ejection fraction can be measured.Valve insufficiencies can be identified [2]
References
  1. HECHT H. H.. Heart Failure and Lung Disease. Circulation [online] 1956 August, 14(2):265-290 [viewed 09 July 2014] Available from: doi:10.1161/​01.CIR.14.2.265
  2. VOELKEL N. F., QUAIFE R. A., LEINWAND L. A., BARST R. J., MCGOON M. D., MELDRUM D. R., DUPUIS J., LONG C. S., RUBIN L. J., SMART F. W., SUZUKI Y. J., GLADWIN M., DENHOLM E. M., GAIL D. B.. Right Ventricular Function and Failure: Report of a National Heart, Lung, and Blood Institute Working Group on Cellular and Molecular Mechanisms of Right Heart Failure. Circulation [online] 2006 October, 114(17):1883-1891 [viewed 10 July 2014] Available from: doi:10.1161/​CIRCULATIONAHA.106.632208

Investigations - Screening/Staging

Fact Explanation
Chest X ray [1] Vertebral defects can be visualised. Also right atrial and ventricular enlargements can be visualised [1]
Echocardiogram [2] Wall thickness,chamber dilatations,ejection fraction can be measured.Valve insufficiencies can be identified [2]
Spirometry [1] Forced Expiratory Volume in One Second (FEV1)/Forced Vital Capacity (FVC) < 0.7 FEV1 >/= 80% predicted [1]
References
  1. WEITZENBLUM E.. CHRONIC COR PULMONALE. [online] 2003 February, 89(2):225-230 [viewed 10 July 2014] Available from: doi:10.1136/heart.89.2.225
  2. HECHT H. H.. Heart Failure and Lung Disease. Circulation [online] 1956 August, 14(2):265-290 [viewed 10 July 2014] Available from: doi:10.1161/​01.CIR.14.2.265

Management - General Measures

Fact Explanation
Life style modifications [1] Stop smoking as smoking further worsen the lung diseases [1]
Control of other co morbid conditions [1] Controlling of diabetes,dyslipidemia etc. as these conditions can aggravate the heart diseases [1]
Treating respiratory failure [2] Patients with kyphoscoliosis and chronic respiratory insufficiency are treated either with home oxygen therapy or ventilation. nocturnal nasal intermittent positive pressure ventilation, plus long term oxygen therapy results in more favourable survival and changes in blood gases and respiratory function than long-term oxygen therapy alone [2]
References
  1. HECHT H. H.. Heart Failure and Lung Disease. Circulation [online] 1956 August, 14(2):265-290 [viewed 09 July 2014] Available from: doi:10.1161/​01.CIR.14.2.265
  2. BUYSE B., MEERSSEMAN W., DEMEDTS M.. Treatment of chronic respiratory failure in kyphoscoliosis: oxygen or ventilation?. European Respiratory Journal [online] 2003 September, 22(3):525-528 [viewed 21 September 2014] Available from: doi:10.1183/09031936.03.00076103

Management - Specific Treatments

Fact Explanation
β-receptor antagonists, inotropes, and diuretics [1] To treat heart failure by reducing afterload [1]
Treatment of the lung disease [2] Oxygen (controlled oxygen), Pulmonary vasodilators (Sildenafil), Inhaled nitric oxide- is a more potent vasodilator than oxygen, Reduction in hematocrit - Phlebotomy is indicated in patients with a severe elevation in hematocrit not responding to long term oxygen therapy [2]
Correction of kyphoscoliosis [3] Back braces [3]
Surgical methods [3] To correct kyphoscoliosis [3] Vertebral column resection for the treatment of severe spinal deformity [4] Vertebral column decancellation for the management of sharp angular spinal deformity [5] Staged corrective surgery for complex adolescent kyphoscoliosis due to contractures [6] Thoracic pedicle screw fixation [7]
References
  1. VOELKEL N. F., QUAIFE R. A., LEINWAND L. A., BARST R. J., MCGOON M. D., MELDRUM D. R., DUPUIS J., LONG C. S., RUBIN L. J., SMART F. W., SUZUKI Y. J., GLADWIN M., DENHOLM E. M., GAIL D. B.. Right Ventricular Function and Failure: Report of a National Heart, Lung, and Blood Institute Working Group on Cellular and Molecular Mechanisms of Right Heart Failure. Circulation [online] 2006 October, 114(17):1883-1891 [viewed 10 July 2014] Available from: doi:10.1161/​CIRCULATIONAHA.106.632208
  2. SHUJAAT A, MINKIN R, EDEN E. Pulmonary hypertension and chronic cor pulmonale in COPD Int J Chron Obstruct Pulmon Dis [online] 2007 Sep, 2(3):273-282 [viewed 10 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2695205
  3. HECHT H. H.. Heart Failure and Lung Disease. Circulation [online] 1956 August, 14(2):265-290 [viewed 09 July 2014] Available from: doi:10.1161/​01.CIR.14.2.265
  4. LENKE LG, SIDES BA, KOESTER LA, HENSLEY M, BLANKE KM. Vertebral Column Resection for the Treatment of Severe Spinal Deformity Clin Orthop Relat Res [online] 2010 Mar, 468(3):687-699 [viewed 10 July 2014] Available from: doi:10.1007/s11999-009-1037-x
  5. WANG Y, LENKE LG. Vertebral column decancellation for the management of sharp angular spinal deformity Eur Spine J [online] 2011 Oct, 20(10):1703-1710 [viewed 10 July 2014] Available from: doi:10.1007/s00586-011-1771-0
  6. TAO HR, HUI H, GUO SZ, XIONG LZ, YE ZX, YAN M, LUO ZJ. Staged corrective surgery for complex adolescent kyphoscoliosis caused by back scalding during the childhood period Eur Spine J [online] 2013 Feb, 22(2):305-309 [viewed 10 July 2014] Available from: doi:10.1007/s00586-012-2519-1
  7. LI G, LV G, PASSIAS P, KOZANEK M, METKAR US, LIU Z, WOOD KB, REHAK L, DENG Y. Complications associated with thoracic pedicle screws in spinal deformity Eur Spine J [online] 2010 Sep, 19(9):1576-1584 [viewed 10 July 2014] Available from: doi:10.1007/s00586-010-1316-y