Ventricular Septal Rupture


Article Author:
Ateeq Mubarik


Article Editor:
Arshad Muhammad Iqbal


Editors In Chief:
Bipin Shah
Matthew Ellison
Jorge Palacios


Managing Editors:
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Frank Smeeks
Kristina Soman-Faulkner
Benjamin Eovaldi
Radia Jamil
Sobhan Daneshfar
Pritesh Sheth
Hassam Zulfiqar
Steve Bhimji
John Shell
Matthew Varacallo
Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Hajira Basit
Phillip Hynes
Kavin Sugumar


Updated:
3/14/2019 5:42:06 PM

Introduction

In 1847, Latham first introduced the diagnosis of ventricular septal rupture (VSR). The interventricular septum divides the ventricular chamber into right and left ventricles.

The interventricular septum consists of 2 parts;

  1. Muscular part: Major, lower, and a thick portion, derived from the bulboventricular flange[1]
  2. Membranous part: Minor, upper, and a thin portion, derived from neural crest cells

A rare, but lethal complication of acute myocardial infarction is a ventricular septal rupture. The condition is rare because of an aggressive approach towards early reperfusion therapy, however, mortality is still high. Any part of the interventricular septum can develop a rupture. The size of the rupture determines the prognosis of the patient. The prognosis is good if the rupture is small and the patient is hemodynamically stable.  

Etiology

The most common cause of ventricular septal rupture is full-thickness (transmural) myocardial infarction in one of the following coronary arteries:

  1. Left anterior descending coronary artery; supplies most of the anterior portion of the interventricular septum responsible for apical VSR.
  2. Dominant right coronary artery; supplies the most inferior portion of the interventricular septum responsible for basal VSR.
  3. Dominant left circumflex artery; supplies the posterior portion of a posterior descending artery arises from the circumflex branch.

Partial thickness infarct such as Non-ST elevation myocardial infarction or unstable angina can also increase the risk of ventricular septal rupture

Epidemiology

Before the invention of thrombolytic and percutaneous intervention, the incidence of ventricular septal rupture was approximately 2%.[2] However, in the current period of thrombolytic and percutaneous intervention, especially with the aim of reducing door to intervention time, the incidence of ventricular rupture has been reduced to 0.31%.[3] According to the Global Registry for Acute Coronary Events (GRACE) study, the rate of ventricular septal rupture in an acute myocardial infarction treated with percutaneous intervention is less (0.7%) than the myocardial infarction in patients treated with thrombolytic therapy(1.1%). [4] This study proved that the rate of ventricular septal rupture is less in patients who received an early thrombolytic or percutaneous intervention.[4] The incidence also depends upon the type of myocardial infarction and is higher in patients with ST-segment elevation associated myocardial infarction (0.9%). Its occurrence in non-ST-segment elevation myocardial infarction and unstable angina is 0.17% and 0.25%, respectively. There is no difference in the rate of ventricular septal rupture based on the location of infarction whether it is anterior infarct or inferolateral infarct.[3]

Pathophysiology

The most common pathological finding of an infarcted septum is coagulation necrosis, which is defined as dry denaturation of proteins due to lack of oxygen as a result of a loss in blood supply. It progresses to thinning and weakening of the septum. This process usually takes three to five days after acute myocardial infarction. The ventricular septal rupture can occur within 24 hours of myocardial infarction due to dissection of an intramural hematoma or hemorrhage into the diseased myocardium.

The primary mechanism behind ventricular septum rupture is physical shear stressors especially at the conjunction of infarct area and normal healthy myocardium. Due to this mechanism, ventricular aneurysm, free wall rupture, or papillary muscle rupture associated with ventricular septum rupture.

Cardiac free wall rupture and ventricular septum rupture after myocardial infarction are similar regarding pathological characteristics. There is a pathological classification regarding free wall rupture which we can use for ventricular septum rupture proposed by Becker.[5] There are three types are as follows:

  1. Type I: Sudden in onset, slit-like tear, within 24 hours
  2. Type II: Subacute, erosion of infarcted myocardium
  3. Type III: Late presentation, aneurysm formation, and rupture, associated with older infarcts

After the establishment of the new connection between right and left ventricle due to ventricular septum rupture, oxygenated blood shunts from the high-pressure left ventricle to the low-pressure right ventricle.

History and Physical

The patient may present with flash pulmonary edema after 1 to 3 days of a stable MI. Severe cases present as the cardiogenic shock.[6] The risk factors for ventricular septal rupture include female gender, increased age, first episode of myocardial infarction, ST-segment elevation myocardial infarction, high GRACE risk score, and chronic kidney disease.[6]. A patient having an acute myocardial infarction may present with hypotension or hemodynamically instability, and this should prompt evaluation for ventricular septal rupture. Characteristic signs the septal rupture include the development of a harsh systolic murmur with a palpable thrill over the precordium. Due to increased right heart flow sometimes there is a loud pulmonic component of the second heart sound, tricuspid regurgitation, or third heart sound.

Evaluation

Two-dimensional echocardiography with Doppler is used to diagnose ventricular septal rupture which shows a flow of blood across ventricular septum.[7] Echocardiogram also demonstrates right ventricular dilatation and pulmonary hypertension due to increased right-sided blood flow. The color Doppler echocardiography is also useful in the evaluation of the anatomical size of a rupture. 

Transoesophageal echocardiography is indicated in the patients in whom it is difficult to get an adequate view of the myocardium, for example in patients who are on a mechanical ventilator or have a large body habitus.

Treatment / Management

An interprofessional team, including an interventional cardiologist and cardiothoracic surgeon, is required to treat the ventricular septal rupture. The ultimate treatment of ventricular septal rupture is a surgical repair.[8] Before surgical repair, it is necessary to restore the circulation in the diseased artery to decrease the hypoxic burden in the infarcted area especially in the cases of right ventricular involvement. 

There are two surgical techniques for the repair of ventricular septal rupture are as follows:

  1. Daggett procedure: Patch over the defect with sutures in both the ventricles (infarct inclusion technique)
  2. David procedure: Patch over the rupture with stitches in the left ventricle only (infarct exclusion technique)

The repair of a posterior ventricular septal rupture is more challenging than anterior rupture due to the proximity of papillary muscles.

If the ventricular septal rupture develops within 24 hours of an MI, surgical intervention is more difficult as it is difficult to differentiate between healthy and newly infarcted tissue; also, the muscle is weak and not able to hold the sutures.

Due to the reason mentioned above, the mortality of surgical intervention within 24 hours of acute myocardial infarction is over sixty percent. In contrast, untreated ventricular septal rupture has a death of 40% to 80%. Late surgical intervention has a good prognosis. Surgical intervention within seven days of this complication has the mortality of 54.1%. On the other hand, surgery after seven days has a death of 18.4%.[9]

Patients presenting with cardiogenic shock need an intra-aortic balloon counterpulsation to reduce afterload and increase cardiac output. 

Percutaneous intervention can repair anterior defects ventricular septal defects less than 1.5 cm in diameter, but this technique is still in an evolutionary phase.[10]

Differential Diagnosis

  • Acute Mitral regurgitation due to papillary muscle rupture
  • Free wall rupture
  • Tricuspid regurgitation
  • Congenital ventricular septal defect
  • Atrial septal defect
  • Acute flash pulmonary edema

Prognosis

The monitoring is essential in these patients. The surgical mortality is also high and requires close monitoring. The surgery should be done after the interactive discussion of the heart team amongst themselves and with the family. The prognosis is favorable if the rupture size is small and the patient is hemodynamically stable. 

Complications

  • Cardiogenic shock[11]
  • Death
  • Ventricular aneurysm
  • Thrombus formation
  • ventricular arrhythmias
  • Free wall rupture

Postoperative and Rehabilitation Care

The patient will require long term cardiovascular rehabilitation and should be enrolled in a supervised exercise program. 

Enhancing Healthcare Team Outcomes

An interprofessional approach to ventricular septal rupture is recommended involving physicians, nurses, and pharmacists working as a team to quickly evaluate and treat the patient.

Ventricular septal rupture is a life-threatening complication of an acute MI. Because of the very high mortality, a multidisciplinary approach is necessary. As soon as the diagnosis is confirmed we should notify the cardiac surgeon. Intensive care unit (ICU) monitoring is vital, and surgery should be undertaken emergently, depending on the hemodynamic status. Most patients require an intra-aortic balloon pump to assist with hemodynamics. Even after surgery, patients develop a whole host of complications including multiorgan failure and persistence of a shunt. When possible, the physician should manage residual shunts with a percutaneous closure device. Despite optimal treatment, the condition carries a mortality of more than 20%.[12][13] (Level V)


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Ventricular Septal Rupture - Questions

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Following a myocardial infarction, a patient develops a loud systolic murmur. The patient has minimal changes in hemodynamics, and his chest x-ray is clear on day 2. However, on day 3, the patient starts to become hypotensive and fails to respond to maximal doses of inotropic drugs. An intra-aortic balloon pump (IABP) is inserted at the bedside. What is the next step in his management?



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Which of the following statements about ventricular septal rupture (VSR) after an MI is inaccurate?



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Which of the following statement about ventricular septal rupture (VSR) is false?



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A 55-year-old male with past medical history of essential hypertension and diabetes mellitus presented with chief complaint of chest pain five days duration. EKG shows ST elevation in inferior leads and labs showed elevated troponins. The patient went for cardiac catheterization and received a drug-eluting stent in right coronary artery. The next day the patient developed lightheadedness, shortness of breath and bilateral pedal edema. Blood pressure is 90/60 mm Hg and heart rate 120 beats per minute. On examination, there is increased jugular venous pressure, bilateral crackles and harsh systolic murmur. What will be the next step in the management?



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A 65-year-old male presented with chest pain and later on diagnosed with ST-elevated myocardial infarction underwent cardiac catheterization, and drug eluting stents have been placed. On the fifth day of post-myocardial infarction patient developed low blood pressure and new harsh systolic murmur. Bedside emergent ultrasound was done showing ventricular septal rupture. What will be the best next step in the treatment of this patient?



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A 70-year-old male with a past medical history of diabetes mellitus, chronic kidney disease, and hypertension recently admitted due to ST-segment elevation myocardial infarction. After five days the patient developed low blood pressure and harsh systolic murmur. Echocardiogram showed ventricular septal rupture (VSR). Intraaortic balloon pump (IABP) has been placed for hemodynamically stability, and cardiac surgery has been called for intervention. How does patient get benefit from IABP device in ventricular septal rupture?



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Ventricular Septal Rupture - References

References

Drazen JM, Expression of Concern: Beltrami AP et al. Evidence That Human Cardiac Myocytes Divide after Myocardial Infarction. N Engl J Med 2001;344:1750-7 and Quaini F et al. Chimerism of the Transplanted Heart. N Engl J Med 2002;346:5-15. The New England journal of medicine. 2018 Nov 8     [PubMed]
Moreyra AE,Huang MS,Wilson AC,Deng Y,Cosgrove NM,Kostis JB, Trends in incidence and mortality rates of ventricular septal rupture during acute myocardial infarction. The American journal of cardiology. 2010 Oct 15     [PubMed]
López-Sendón J,Gurfinkel EP,Lopez de Sa E,Agnelli G,Gore JM,Steg PG,Eagle KA,Cantador JR,Fitzgerald G,Granger CB, Factors related to heart rupture in acute coronary syndromes in the Global Registry of Acute Coronary Events. European heart journal. 2010 Jun     [PubMed]
Becker AE,van Mantgem JP, Cardiac tamponade. A study of 50 hearts. European journal of cardiology. 1975 Dec     [PubMed]
Papalexopoulou N,Young CP,Attia RQ, What is the best timing of surgery in patients with post-infarct ventricular septal rupture? Interactive cardiovascular and thoracic surgery. 2013 Feb     [PubMed]
Attia R,Blauth C, Which patients might be suitable for a septal occluder device closure of postinfarction ventricular septal rupture rather than immediate surgery? Interactive cardiovascular and thoracic surgery. 2010 Nov     [PubMed]
Wenink AC, Embryology of the ventricular septum. Separate origin of its components. Virchows Archiv. A, Pathological anatomy and histology. 1981     [PubMed]
Huang SM,Huang SC,Wang CH,Wu IH,Chi NH,Yu HY,Hsu RB,Chang CI,Wang SS,Chen YS, Risk factors and outcome analysis after surgical management of ventricular septal rupture complicating acute myocardial infarction: a retrospective analysis. Journal of cardiothoracic surgery. 2015 May 4     [PubMed]
Boettler P,Claus P,Herbots L,McLaughlin M,D'hooge J,Bijnens B,Ho SY,Kececioglu D,Sutherland GR, New aspects of the ventricular septum and its function: an echocardiographic study. Heart (British Cardiac Society). 2005 Oct     [PubMed]
Misawa Y, Off-pump sutureless repair for ischemic left ventricular free wall rupture: a systematic review. Journal of cardiothoracic surgery. 2017 May 19     [PubMed]
Glancy DL,Khuri BN,Mustapha JA,Menon PV,Hanna EB, Myocardial infarction with ventricular septal rupture and cardiogenic shock. Proceedings (Baylor University. Medical Center). 2015 Oct     [PubMed]
Malhotra A,Patel K,Sharma P,Wadhawa V,Madan T,Khandeparkar J,Shah K,Patel S, Techniques, Timing & Prognosis of Post Infarct Ventricular Septal Repair: a Re-look at Old Dogmas. Brazilian journal of cardiovascular surgery. 2017 May-Jun     [PubMed]
Premchand RK,Garipalli R,Padmanabhan TN,Manik G, Percutaneous closure of post-myocardial infarction ventricular septal rupture - A single centre experience. Indian heart journal. 2017 Apr     [PubMed]

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